Download Jean Bernard Causse, MD - American Otological Society

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AMERICAN OTOLOGICAL SOCIETY, INC.
2002
VOLUME 90
ONE HUNDRED THIRTY FIFTH ANNUAL MEETING
BOCA RATON RESORT&CLUB
BOCA RATON, FL
MAY 10-11
2002
Legend for Photo AOS 2002
First Row: Left to Right
D. Tucci, K. Doyle, S. Wetmore, L. Parnes, G. Lesinsky, R. Chole, M. Paparella, J.
Gulya, H. Konrad, S. Kinney, L.Okdvist, J. Neely, B. Wellimg, C. Shelton.
2nd. Row: Left to Right
T. Haberkamp, S. Levine, J. Hough, R. Ruggles, B. Gantz, W. Moretz, M. McGee, R.
Bellucci, W. Montgomery, B. Hirsch, B. Lonsbury-Martin, J. Dickens, J. Nedzelski.
3rd. Row: Left to Right
T. Murphy, W. Adkins, H. Silverstein, J. Harris, G. Matz, T. VanDewater, R. Amedee, A.
Eviatar, J. Farrior, L. Duckert, F. Rizer, W. Lippy.
4th. Row: Left to Right
D. Weider, G. Singleton, R. Kohut, J. Grote, M. Arriaga, C. Beatty,
Friedman, T. Eby,
E. Chiossone, A. Shuring, N. Wendell-Todd.
R.
5th. Row: Left to Right
H. Brodie, A. Sismanis, J. Farmer, P. Antonelli, A. Lalwani,H. Jenkins, D. Kamerer, P.
Weber, J. McElveen, D. Pappas,
, D. Lim, O. Black, C. Luetje, J. Pulec.
6th. Row: Left to Right from center
J. Niparko, N. Coker, A. Kumar, P. Hammerschlag, E. Monsell, J. Rubinstein, M.
Hannley, G. Spector, G. Hughes, G. Gates, A. Arts.
Award of Merit Presentation to
Gregory J. Matz
By
C. Gary Jackson
The most senior past president of the Society views the Award of Merit process with some level of
ambivalence. On the one hand, it is his or her last official executive act and therefore constitutes his exit
from the Council. My service to the Society on the Council has clearly represented the capstone of my
professional life. It is an experience that I will always cherish. I would like to express my sincerest
gratitude for your support and significant cooperative spirit during my tenure. But, on the other hand, in
this era of hate, terror and terrorism, it is indeed fortifying to recognize and honor merit and all that this
term inspires. The Award of Merit Committee, I think, has selected a worthy recipient with whom you
will be justly pleased. At this time, I would like to acknowledge the Award of Merit Committee and
express my gratitude for all their deliberative effort. So let's get to it!
Our story begins in March of 1937 in Chicago. Born premature, our recipient's mother, an RN cared for
him in what had to be the city's first neonatal ICU. Born into the inner city, in an area known as
Bucktown, this was a predominantly hardworking, Polish enclave of the city of Chicago. Now gentrified,
at the time people were very anxious to leave. Most spoke the Polish language, a language he never
learned, but I have it on excellent authority that our recipient learned and knew, very well, all of the swear
words. It has been claimed that he's never had grass around his house until the age of 26! Ma and pa
owned a grocery store and lived in a "3-flat". The business was on the first floor, grandmother occupied
Floor 2, and the family Floor-3 .His whole family lived within two blocks of this location. He was
significantly, therefore, mentored and couldn't get away with much. Even at parochial school it was tow
the line! I assure you, having to deal with one of these, sister, oh no you don't at 5-feet, and 225-pounds
having a bad day will incentivise you to be successful like no other force in nature. This ensured a
virtually angelic youth.
Our Awardee had sartorial integrity. There were early standards as depicted here in a photograph with his
dapper father. These standards were clearly maintained as we see in this photograph with his brother at
about age 12. Born premature, there were some visual color-blind issues. To this day he is known to
appear with different color socks, mismatching ties, etc. But, being the boss, very few people brought this
to his attention. I also have it on good authority that while in the Air Force, in the late 1960's at Brooks
Air Force Base, he was the only Major, in his dress blues who would be seen regularly in his penny
loafers. He has also been known to have color coordinated trip suitcases. His wife would pre-label all
items of clothing and, therefore, coordinate them. This seemed to work quite well until one day getting
out of a taxi cab his suitcase fell open discharging the labels to the will of the wind and confounding his
fashion sense.
This is clearly a territorial animal, a creature of the city of Chicago. He lived for baseball, street ball and
to this day, and I venture to say to the day he dies, can be counted amongst those dyspeptic and morose
Chicagoans known as Cub fans. He is a creature of the city and of his high school which demonstrated
and meant a great deal of territorial integrity to him. To this day, every applicant or acquaintance he
meets, they appear to be disarmed by the question, "Where did you go to high school".
He soon began to flirt with independence. His family never had or could afford a car. Every transportation
he either made by bicycle or the "El". As a matter of fact, all his educational choices were based upon
location and travel distance by bus or "El". Witness his alma mater of DePaul, Loyola, and the University
of Chicago, all only one bus or train ride away. His independence began, however, to blur into the
confines of responsibility .His father died when he was 16. Mom sold the store and returned to her duties
and profession as a nurse. The boys had to fend for themselves and I again have it on good authority that
he and his brother led an "adventuresome" life. His brother died prematurely at age 33 leaving a wife with
a small child.
He finally owned a car in residency, but all the independence was just too much!
In June he opened the trunk of his car to find one-hundred addressed Christmas cards stamped from the
previous year. He was said to have fallen to his knees to clutching his head in both hands claiming, "1
need help". After a complicated courtship, he eventually proposed to a woman to whom he referred as
"GU': Geographically undesirable. She lived in the suburbs! The rest is history.
His career as a mentor and teacher at the University of Chicago and later as the Chair at Loyola for
eighteen years constituted the concept of merit. His service to this Society is virtually unparalleled. All of
this was achieved with his partner in life, his wife Judy. He was always a serious student of his
profession. His advice and decisions were always secure, seldom wrong, but never in doubt!
Now recently retired from the Chair at Loyola University, the passions of his life include his family,
particularly those newest members of the family, his grandchildren. He is an avid sailor and spends as
much time on his boat as possible. And, oh yes, there's Chicago, I feel his passion forever.
Ladies and gentlemen, I introduce to you this year's recipient of the Award of Merit of the American
Otological Society, Dr. Gregory Matz.
2002 PRESIDENTIAL ADDRESS
RICHARD A. CHOLE, MD PhD
One of the prerogatives of the President is to give a short Presidential Address and I had
debated about what I should say at this address being focused as a physician scientist on the
science under-girding the specialty. I thought that would be my focus but there are other
pressing issues facing otology and otolaryngology. I just thought I would give you some of my
comments about the movement in audiology that is occurring as most of you probably heard
about this morning from Dr. Fabrey.
I’d like to talk to you about the future of audiology,
some perspectives on the new AUD movement and what it
means generally, what kind of a movement it is and what it
means to us. This is from the AAA about their answer as to
why have an AUD. The scope of practice of audiology has
expanded substantially since the 1960’s when a master’s
degree rather than a bachelor’s degree was required for entry
into practice. It has become necessary to increase the
academic and clinical training requirements from a master’s
degree to a professional doctorate for entry into the profession.
Professional organizations have indicated that professional
doctorate; the AUD will be the entry-level degree by 2012.
This is basically the marching order and the march has begun.
A few things about the AUD from the AAA and other
professional sources: The AUD is not an academic degree; it’s
a clinical professional degree like those in the fields of
medicine and dentistry. Students go to the best schools
because those graduates will flourish in the doctoring world. The AUD is here to stay. I’d like
to point out a couple of factors here. The AUD is a clinical degree and there is some concern on
my part and many people’s part as to where do the academic development come in a clinical
training program like this. Will perspective PHD researchers in the audiology field choose the
AUD instead of choosing a research degree, which the AUD is not?
The other point is the statement that this is a doctoring profession and a doctoring degree.
This is the difference between current master’s level training and the AUD. The length of
training is lengthened from two to three or sometimes four years. Credits are about the same.
The scope of information is roughly the same with the addition of emphasis on cochlear implant
rehabilitation and some neuro-monitoring and neurophysiology as well as vestibular testing
being integrated into the programs, not only vestibular testing but vestibular rehabilitation as
well. So, the scope of the AUD has expanded beyond the MS slightly. These programs started
only a few years ago but this is from a recent website 39 and counting. These are programs that
are either in place or are going to be in place and these are not by small and insignificant
University but many which you represent here who are participants in this movement.
This is from one of their websites as to what will the AUD do for me for the audiologist?
And this is what the promise of the AUD is. First, autonomy graduates will be able to provide
hearing services independently. Flexibility, the graduates will have flexibility to provide
services to an entire range of patients. That is a different focus than the prior focus. Higher
incomes, individuals who hold doctoral degrees can expect higher incomes and public
recognition and serving the public. So the AUD movement has certain expectations which are
well described. A number of these programs currently, probably most of the programs are run
with a distance learning paradigm, that is you can get the degree and accumulate credits and
actually be awarded the degree without ever really attending a University or a School.
Here some of the schools that are offering distance learning for an AUD and I’d like to
show you some details on one of them just as an example so you get a feel for this. The
Pennsylvania College of Optometry has a School of Audiology. This is from their website
“Welcome to the Pennsylvania College of Optometry, School of Audiology AUD’s distance
education website. AUD on line is a bridge program that allows audiologists to earn an AUD
degree without leaving their practices. AUD online is reasonably priced and offered 100% on
the internet.” Here’s an example of some of the courses of AUD online: anatomy and
physiology and the ECOG, anatomy and physiology ENG, otoscopy and cerumen management,
business management, vestibular rehabilitation and medical surgical treatment of the ear. I’m
not sure how they teach medical and surgical treatment of the ear online but that’s what’s
happening. Here are expectations of the AUD movement, principally the independent practice
potential. Independent practices in hearing evaluation and hearing aids. There’s a lot of interest
among the AUD’s and the AUD schools that routine testing may not really be done by AUD’s
but rather by technicians that the AUD's will employ or train. I’ll get back to that in a minute.
Clearly this is a doctoring profession that they will be addressed as doctor. Balance disorders are
being incorporated into the realm of the AUD including diagnostic testing, diagnosis and
treatment in rehabilitation in balance disorders. Neuro-monitoring is also within the scope and
the expectation of higher compensation. So really one of the big questions here is once the AUD
movement has been full-blown and I guarantee to you that it will be and the master’s degree
level will vanish within the next few years, whose going to fill the gap of doing diagnostic
audiology and hearing testing? There are needs for otolaryngologists to have someone working
in their offices. There are needs for schools to have audiologists and AUD’s themselves are
going to want to hire somebody to do hearing testing so these are some of the proposals. The
oto-tech proposal that technicians can be trained and a technician course something like the
Academy is proposing, an associates degree that would be given in an institution of higher
learning where someone would earn an AA for learning basic hearing testing, maybe a
continuation of the master’s degree audiologist. The way that the snowball is going, it’s my
feeling that those will really disappear.
Another suggestion that we are going to make is the idea of a bachelor of science in
Audiology be resurrected and that quality training programs and basic audiology be offered on a
bachelors science level so that well trained individuals will be able to do the gamete of
diagnostic audiology was done before the master’s degree gathered momentum.
This is a topic of discussion. We can’t really be complacent about this particular issue
because the train has left the station. So what can we do? Well here are a few thoughts about
our response to this movement. First of all I think we should support the training and
development of another level of audiologists. This might be an oto-tech, an audiology technician
or maybe go back to training bachelors of Science audiologists in addition to the AUD. I think
it’s imperative that we improve training in our residency programs in audiology. Speaking as a
member of the American Board of Otolaryngology, its appalling the level of training that an
average otolaryngology resident has when he or she comes to take the boards. This must change.
I think that can change by challenging the residency review committees to really require
audiology training in the residency programs as it was when I was a resident when there was a
much bigger core of audiology training than what we have now. Also challenge the AB OTO to
increase emphasis on audiology and hearing aids and audiometric testing in both the certifying
examination and the maintenance of certification. Then I would also challenge the University’s
and challenge you at your University’s to resist the idea of distance learning and the idea of
unearned degrees. These degrees are being grandfathered as well
as and I think that’s an extremely bad trend. The AUD is here to stay and I think if it’s done in a
responsible and professional manner, these individuals will be well trained and served well. I
think we also have a mandate to indeed do this properly and we all need to step up to the plate
and do our role in audiometric training. Thank you.
GUEST OF HONOR PRESENTATION
DAVID A. LIM
PRESIDENTIAL CITATION
MICHAEL M. PAPARELLA
Scientific Session
May 10, 2002
Acoustic Neuroma
Vestibular Schwannomas Growth Rates in NF2
Natural History Consortium Patients
William H. Slattery, MD, Mark L. Oppenheimer, MS
Laurel M. Fisher, PhD
Objective: To examine the growth patterns of vestibular schwannomas (VS) in NF2 patients.
Study design: Retrospective case review.
Setting: Multiple tertiary centers across the world (US, England, Germany, and Japan) participating in NF2 Natural
History Consortium.
Patients: 291 retrospective MRI exams from 88 NF2 patients were examined, resulting in a total of 579 data points
for 175 VS. Short-term analyses were restricted to those VS with: 1) at least two data points in time, 2) at least 3
months follow-up, 3) no VS surgery prior to end of follow-up. After these exclusions, 343 data points for 94 VS
(from 58 NF2 patients) remained. Long-term analyses had the additional restrictions of: 4) at least 4 data points, 5)
at least 18 months of follow-up. 165 data points for 27 VS (from 21 NF2 patients) remained for long-term analyses.
Intervention(s): None
Main Outcome Measure(s): The primary outcome measure was greatest anterior posterior dimension. In addition,
changes in greatest medial lateral and greatest diameter were analyzed.
Results: For short-term analyses (n=94 VS), VS at earliest MRI had a mean anterior posterior measurement of
1.1cm (sd=0.8), while the mean at last MRI was 1.3cm (sd=0.9). For long-term analyses (n=27 VS), VS at earliest
MRI had a mean anterior posterior measurement of 1.0cm (sd=0.5), while the mean at last MRI was 1.3cm
(sd=0.7). Further analyses will be reported, including results stratified by tumor size at earliest MRI.
Conclusions: In general, VS from NF2 patients were stable during this analysis. Prospective standardized studies
are needed to determine accurate growth rates
William H. Slattery, M.D.
House Ear Institute
2100 West 3rd St.
Los Angeles, CA 91011
The Efficacy of Corticosteroids in Restoring Hearing
Loss in Patients with Acoustic Neuromas
Anna Aronzon, MD, Douglas C. Bigelow, MD
Michael J. Ruckenstein, MD, MSc, FRCSC
Objective: To evaluate the role of corticosteroids in restoring hearing loss in patients undergoing conservative
management of acoustic neuromas.
Study design: Prospective cohort.
Setting: Tertiary referral center.
Patients: Seven patents who presented with acute hearing loss secondary to an acoustic neuroma.
Intervention: Oral Prednisone (1 mg/kg x 1-2 weeks) within a week of onset of hearing loss.
Main outcome measures: Audiometric assessment at the time of presentation and after corticosteroid therapy.
Results: All seven patients had pure tone reception threshold improvements ranging from 10 to 50 dB in at least two
frequencies or more. Speech discrimination thresholds improved after therapy in all seven patients with gain of 4 to
38 percentage points.
Conclusions:
1. Patients with acoustic neuromas who are being managed with a "watchful waiting" approach should receive
corticosteroid treatment at the first signs of hearing deterioration.
2. A retrocochlear work-up is mandated in patients presenting with sudden hearing loss even if it responds to steroid
treatment.
3. Older data documenting the efficacy of corticosteroids in the treatment of sudden hearing loss may need to be reevaluated, as many of these studies were conducted prior to the advent of MRI scanning.
4. These data are the first to document the efficacy of corticosteroid treatment in reversing acute hearing loss caused
by acoustic neuromas.
Michael J. Ruckenstein, M.D.
Dept. of Otorhino
Hospital of the University of Pennsylvania
3400 Spruce Street, 5 Ravdin laryngology
Philadelphia, PA 19104
CSF Leakage after Acoustic Neuroma Surgery: A Comparison of the
Translabyrinthine, Middle Fossa, and Retrosigmoid Approaches
Samuel S. Becker, BA, Robert K. Jackler, MD
Lawrence H. Pitts, MD
Objective: To determine whether or not the choice of surgical approach affects the rate of postoperative
cerebrospinal fluid (CSF) leakage in patients who underwent surgical resection of acoustic neuroma.
Study Design: Retrospective chart review.
Setting: Tertiary referral center.
Patients: 300 patients who underwent surgery for acoustic neuromas were selected by consecutive medical record
number until 100 resections via each surgical approach (translabyrinthine (TL), middle fossa (MF), retrosigmoid
(RS)) had been gathered.
Main Outcome Measures: Surgical approach utilized, CSF leak incidence, tumor size, patient age.
Results: Post-operative CSF leak of any severity was found in 13% of TL, 10% of MF, and 10% of RS patients.
These difference in the rate of CSF leakage were not statistically significant (p=0.72). The majority of leaks were
managed conservatively with fluid and activity restriction often accompanied by a period of lumbar subarachnoid
drainage. There was a need to return to the operating room for a definitive procedure in 5% of TL, 2% of MF, and
4% RS patients; again not statistically different among the
approaches (p=0.51). Tumor size was not correlated with CSF leak rate (p=0.13). Patient age, for patients older than
50 years, was suggestive of increased odds of CSF leak (p=0.07).
Conclusion: Neither surgical approach nor tumor size affects the rate of post-operative CSF leakage or the
necessity of managing a leak with a return to the operating room. The odds of incurring post-operative CSF leaks
may increase with age beyond 50 years.
Samuel S. Becker, B.A.
144 Hugo Street #3
San Francisco, CA 94122
DISCUSSION SESSION #1, Papers 1-3.
DR. RICHARD CHOLE:
These papers, the Presidential Address and the Guest of Honor Address are up for discussion, so
are there any comments or questions?
DR. DAVID EISENMAN, WASHINGTON DC:
On the last paper, did the patients and different approaches have different postop treatment
dosage or duration of steroid administration?
DR. SAMUEL BECKER:
We don’t routinely use steroids except for very large leaks.
DR. ARUN GADRE, GALVESTON,TX:
You spoke about diffensins. My understanding was that diffensins have very little effect against
gram negatives in general and in pseudomonas in particular. Have you looked at that?
DR. RICHARD CHOLE:
David, did you get the question? The question about diffensins and their activity against gram
negatives?
DR. DAVID LIM:
That is not true our data shows that it is not as strongly active against gram positive but they are
quite comparable.
DR. ARUN GADRE, GALVESTON, TX:
Because there’s a lot in the literature with regards to bovine mastitis and looking at different
organisms particularly with respect to diffensins, there’s a huge body of literature that you are
aware of. My understanding was that for pseudomonas it’s not very good.
DR. DAVID LIM:
We have not tested pseudomonas but we are largely interested in pseudo-pathogens that I
described and the dramatic one was against the moroxilla, then streptococcus pneumonia, and
hemophilus. This maybe has to do with the way that the human better diffensins are prepared, so
I think from one study to the other when it’s right to compare it, you have to know exactly what
the preparation is.
DR. RICHARD CHOLE:
Thank you, other questions or comments? Okay I would like to remind the members that the
AOS photograph will be taken this afternoon just after the end of these sessions and it will be in
the Spanish patio, that’s where all the birds are over as you walk through and we will remind you
again of that. I’d like to encourage you to see the exhibitors across the way and we will
reconvene here at 3:15 for the next session on Implantable Devices. Thank you.
Implantable Devices
Rehabilitation of Unilateral Deafness: Comparison of the BAHA
Implantable Hearing Device with CROS Amplification
John K. Niparko, MD, Lawrence R. Lustig, MD
Kenneth M. Cox, MA, CCC-A
Objective: Hearing in only one ear imposes constraints under many listening conditions. We compared the speech
recognition effects of a semi-implantable bone-conductor (the Entific BAHA device) with conventional contralateral
routing of signal (CROS) amplification in adults with unilateral deafness.
Design: Prospective trials of subjects with unilateral deafness using hearing-in-noise (HINT) and source
identification (SAINT) testing.
Setting: Tertiary referral center; outpatient surgical and audiological services.
Patients: Adults with unilateral deafness (SRT>90dB; S.D.<20%) after acoustic neuroma excision (n=7) or sudden
SNHL (n=3). Entry criteria included normal hearing in the contralateral ear (SRT<25dB; S.D.>80%).
Intervention: Subjects were fitted with CROS amplification devices for one month and tested with CROS before
(mastoid) implantation of the deaf ear, fitting, and testing for BAHA.
Outcome measures: 1) Subject assessment of experience with their devices and patterns of use; 2) speech
discrimination in quiet and in noise under conditions of noise-front, noise-to-normal-ear, and noise-to-deaf-ear using
HINT protocols; and 3) source localization tests with SAINT.
Results: There was consistent satisfaction with BAHA implantation and amplification, and poor acceptance of
CROS amplification. Relative to baseline, CROS and BAHA produced significantly better speech recognition in
noise under most conditions. BAHA enabled significantly better speech recognition than CROS in the noise-todeaf-ear condition. Sound localization was poor at baseline, and with both BAHA and CROS.
Conclusions: Preliminary data indicate that the BAHA overcomes head shadow effects in unilateral deafness.
BAHA improves speech recognition in noise based on filtering characteristics of the device and interaural signal
transfer. BAHA offers advantages over CROS by avoiding occlusion of the normal hearing ear canal, thereby
averting interference of speech signals. BAHA provides a superior approach to CROS in the auditory rehabilitation
of unilateral deafness as revealed by initial patient experience and
performance.
Supported by the Dexter F. and Dorothy H. Baker Foundation, equipment support provided by Entific Medical
Systems.
John K. Niparko, ,M.D.
Johns Hopkins Hospital
JHOC-6223
601 N. Caroline St.
Baltimore, MD 21287-0910
The Use of Full-Thickness Skin Grafts for the Skin-Abutment
Interface Around Bone-Anchored Hearing Aids
Mary C. Snyder, MD, Gary F. Moore, MD
Perry J. Johnson, MD
Objective: To review the complication rate encountered with the use of full-thickness skin grafts to obtain a viable
skin-abutment interface around bone-anchored hearing aid (BAHA) implants.
Study Design: Retrospective chart review
Setting: Tertiary referral center
Patients: Fifteen patients who underwent BAHA placement over a four-year period.
Intervention: Each percutaneous titanium implant and abutment was placed into the temporal bone the standard
Branemark technique. Eight of these procedures were performed in two stages, and seven were done as single stage
procedures. In all cases, the skin-abutment interface was established by use of a full-thickness skin graft inset
around the implant.
Main Outcome Measure: Complications associated with the skin grafts were noted, as well as any additional
procedures needed for revision of the skin-abutment interface.
Results: Seven patients (46.7%) experienced loss of the full thickness skin graft around the abutment. Three of
these seven had complicating medical factors associated with impaired wound healing: two with diabetes mellitus,
one of whom was also a smoker, and a third patient who was receiving inhaled steroids for treatment of asthma. Of
the seven patients who lost skin grafts, two healed by secondary intention, two underwent repeated full thickness
skin grafting, and three underwent galeal rotation flaps with split-thickness skin grafting. No patient experienced
loss of the implant.
Conclusion: The use of full-thickness skin grafts for establishment of the skin-abutment interface around BAHA
implants is associated with a high rate of graft loss. Alternative methods of establishing the skin-abutment interface
should be considered, especially in high-risk patients.
Mary C. Snyder, M.D.
981225 Nebraska Medical Center
Omaha, NE 68198-1225
Long-term Results in the First 100 U.S. Recipients of the
Vibrant Soundbridge Implantable Middle Ear Device
Thomas J. Balkany, MD, Charles Luetje, MD
The Vibrant Soundbridge Clinical Investigators Group
Objective: Implantable middle ear devices have been available since August 2000 for treatment of mild-to-severe
sensorineural hearing loss. The objective of this study was to evaluate long-term (12-48 months postoperative)
safety and performance in the first 100 recipients in theUnited States.
Study design: This was a multicenter, prospective, single-subject, repeated measures study.
Setting: The study was completed at 14 tertiary care hospital and outpatient facilities.
Patients: 100 adults with mild-to-severe sensorineural hearing loss were given a battery of tests including otologic
examination, audiometric testing, and self-assessment, prior to implantation with the VSB, and then at postoperative
intervals up to 48 months.
Intervention: Subjects were implanted with a Vibrant Soundbridge (VSB).
Main Outcome Measures: Air, bone, speech and admittance testing measured hearing improvement over time.
Patient satisfaction was measured with a validated self-assessment metric, Profile of Hearing Aid Performance
(PHAP) and complications were analyzed from review of a computerized data base.
Results: Patients maintained statistically significant increases in functional gain compared to their hearing aids over
time. Speech recognition scores were similar to those with hearing aids. Self-assessment of satisfaction and benefit
in different listening situations (PHAP) indicated sustained improvement over time. No clinically significant
decrease in residual hearing or other major complications were caused by the intervention.
Conclusions: This study demonstrates safety and efficacy of the VSB over a period of 12-48 months.
Thomas J. Balkany, M.D., FACS
Dept. of Otolaryngology
University of Miami School of Medicine
Box 016960 (D-48)
Miami, FL 33101
Second Language Capabilities in Children with Cochlear Implants
Susan Waltz man, PhD, Noel Cohen, MD, Amy Robbins, MS
Janet Green, MS, Yael Bat-Chava, PhD
Objective: The development of oral language in children using cochlear implants is dependent on factors including
age at implantation, speech perception abilities and intervention approach. Although baseline achievements have
been established, ceiling attainment levels of this group have yet to be explored. One indicator of a higher capability
level would be the ability of implanted children to learn to communicate orally using a second language. The
purpose of this research was to 1) explore the feasibility of children with cochlear implants developing oral fluency
in a language in addition to English and 2) categorize the levels of speech perception and linguistic competence
necessary for children to learn a second language.
Study design: Prospective study of children fulfilling the inclusion criteria.
Setting: University Medical Center.
Patients: 20 profoundly hearing impaired children who were exposed to a spoken language in addition to English.
Interventions: Cochlear implantation at age 3 or younger.
Main Outcome Measures: The subjects were evaluated preoperatively and at regular intervals postoperatively
using standard speech perception and receptive and expressive language measures.
Results: Results indicate that some children using cochlear implants are able to achieve fluency in a second
language. Statistically significant correlations exist between level of auditory skills, linguistic competence in English
and the ability to learn a second language.
Conclusions: High levels of achievement including the learning of a second spoken language are possible following
cochlear implantation. Variables include the existence of handicapping conditions, type of therapeutic intervention
and educational placement.
Susan B. Waltzman, Ph.D.
NYU Cochlear Implant Center
660 First Avenue
New York, NY 10016
Telephone Use and Understanding in Patients with Cochlear Implants
Jeffrey S. Adams, MD, Gregory W. Pippin, MD
M. Suzanne Hasenstab, PhD, FAAA, Aristides Sismanis, MD
Objective: To determine the number of cochlear implant patients who use the telephone independently, require
some form of assistance, or do not use the telephone. To assess telephone understanding by patients who use the
telephone independently, and determine characteristics that contribute to successful telephone communication.
Study design: Retrospective clinical study.
Setting: Tertiary referral center.
Patients: Ninety-five adult and pediatric patients (born in or before 1991) at our institution using cochlear implants
for at least 6 months.
Intervention(s): A mailed survey assessing telephone communication was used to group subjects according to
telephone use. Response frequencies were compared between groups for questions regarding patient demographics,
clinical and audiologic data, cochlear implant device characteristics, and telephone use. Independent telephone
users were eligible for testing with open-set sentences presented via telephone. Statistical analysis was used to
correlate performance with patient characteristics and device factors.
Main outcome measure(s): Results of the completed survey and the CID Telephone Sentences Test.
Results: A 91% (N=86) survey response rate was achieved. Forty-two percent of respondents indicated
independent telephone use, 14% reported adapter or code-assisted telephone use, and 44% had no telephone use. Of
the independent users, 94% (N=34) participated in the telephone testing task. The average performance score was
67%. Variables associated with independent telephone use and proficient telephone understanding is presented.
Conclusions: Results suggest that many cochlear implant patients use the telephone for daily activities without
assistive devices or relay services. Patient characteristics and factors related to cochlear implants can impact
successful telephone use and understanding.
IRB Approval Number: 1677
Jeffrey S. Adams, M.D.
VCU - MCV Campus
P. O. Box 980146
Richmond, VA 23298
Electrical Suppression of Tinnitus
Jay T. Rubinstein, MD, PhD, Richard S. Tyler, PhD
Carolyn J. Brown, PhD, Abigail Johnson, MA
Cynthia Bergen, BA
Objective: Tinnitus is a widespread clinical problem with multiple treatments but no cure. A cure for tinnitus would
restore the perception of silence. One plausible hypothesis for the origin of tinnitus associated with sensorineural
hearing loss is that it is due to loss or alteration of the normal spontaneous activity in the deafferented regions of the
cochlea. Electrical stimulation of the cochlea with high-rate pulse trains has been demonstrated to produce
spontaneous-like patterns of spike activity in the auditory nerve. We hypothesize that such stimuli might represent
an effective treatment for tinnitus.
Study design: Single-blind trial using each subject as their own control.
Setting: Outpatient clinic in tertiary referral center. Patients: Eleven volunteer human subjects with bothersome
tinnitus and high-frequency sensorineural haring loss.
Interventions: Myringotomy and temporary placement of a round window electrode was performed. High-rate
pulse train stimuli were presented at various stimulus intensities.
Main outcome measures: Tinnitus and stimulus perception were scaled by the
subject without feedback of the stimulus condition. Results: Five of eleven subjects showed substantial or complete
tinnitus suppression with either no perception or only a transient perception of the stimulus. Three showed tinnitus
suppression only in association with the perception of the stimulus. Three showed no effects on tinnitus.
Conclusions: The results are promising and support further research to develop a clinically useful intervention for
tinnitus associated with high-frequency sensorineural hearing loss.
Supported by Braintronics, Inc. and the Tinnitus Research Consortium.
IRB#199810088
Jay T. Rubinstein, M.D., Ph.D.
Dept. of Otolaryngology
200 Hawkins Drive
Iowa City, IA 52242
Discussion Session Papers 4-9
DR> RICHARD CHOLE
We have some time for some questions and answers. If you come up with a question, please state
your name and address the paper you want to discuss, go ahead.
DR. MICHAEL RUCKENSTEIN, PHILADELPHIA:
Dr. Rubenstein can you describe the differences in your stimulus parameters as those defined by
the old Aran data done in the 70’s using electrical stimulation for tinnitus suppression?
DR. JAY RUBENSTEIN:
In one word, rate. The idea here is that we know these high rates of stimuli, specifically this
particular range of three to five thousand pulses per second, produces spontaneous-like activity
in the differentiated auditory nerve. If the loss of spontaneous activity is one mechanism for
tinnitus, then in people who have that, you would expect to be able to suppress their tinnitus with
those sorts of stimuli.
DR . CHARLES LUETJE, KANSAS CITY :
For Dr. Rubenstein. In the mid-1980’s we did some acute studies that is unpublished data where
we put a 3M House device electrode against the round window through a tympanotomy and then
for a series of minutes, hours, I’ve forgotten what it was, with 3M equipment put square waves,
round waves, circular waves, sine waves. Four of those thirteen patients had some alteration of
their tinnitus. My question to you is how might that rate varied from what you are doing here?
DR. RUBENSTEIN:
I can answer that by saying when I first presented the idea of doing this at the neuroprosthesis
workshop a few years ago, Terry Ambrecht’s response was “Gee that’s sounding an awful lot
like a fifteen kilohertz carrier”. Part of what motivated this work is a long history of using high
rate, high frequency sinusoidal stimuli in attempts to suppress tinnitus with variable results. So
the goal here was to try, knowing that this particular range of frequency produces a physiologic
pattern of activity where there are good reasons to think this is associated with certain kinds of
tinnitus, to find out whether it really does.
DR. STEVEN CHEUNG, SAN FRANCISCO, CA
Question for John Niparko. John, a question for you about the directional hearing capacity of
patients with BAHA. The question is: was the condition where you would occlude the only
hearing ear and test the directional hearing capability of patients with BAHA only input used in
your study?
DR JOHN NIPARKO:
I didn’t catch the last phrase.
DR. STEVEN CHUNG, SAN FRANCISCO, CA
Was the condition where the better hearing ear is occluded and the task is to identify the sound
source through the BAHA, the only condition used?
DR.NIPARKO
:
Through the BAHA only, no we didn’t look at that condition. In fact this is a rather rigorous test
because everything is posterior to the patient. There is likely going to be localization tests that
are less stringent where the patients may in fact function better. When you talk to these
individuals, you get the sense that is likely to be the case, because they feel they have some
localization we were not able to show with the tests.
DR. STEVEN CHUNG, SAN FRANCISCO, CA
It might be interesting to see if there is some systematic distortion in terms of localization of
sounds in space with BAHA only and it might be a problem where you have divergence of
representation of space in these two modes of hearing.
DR. NIPARKO:
It’s entirely likely. The hypothesis that you have raised is that because there are low pass filter
characteristics of the skull base that there is going to be a distortion in terms of timing and
frequency cues. That’s going to interfere with precise localization although some degree of
localization may be still possible.
DR. RICHARD CHOLE
Are there any other questions?
DR. RICHARD CHOLE.
A question for Dr. Balkany. Could you comment on device failures or redo complications in the
series?
DR. TOM BALKANY:
In the series there were four non-users. There were also four patients whose hearing results were
substandard and who received middle ear explorations. In that
group, working transtympanically, there was an attempt made to recrimp the device on the incus
and that was successful on improving the hearing results in two. There was one patient who was
explanted because of a feeling of fullness or stuffiness as well. That was probably due to some
bone dust, which was left in the area of the facial recess in which formed a little cast around the
FMT.
DR. RICHARD CHOLE.
Were there no device failures?
DR. TOM BALKANY:
The data that I presented was with the second generation device and there were no device
failures in that group. At the three-month data, those of you who follow this may recall there
were some device failures at the stress relief point similar to what we saw with earlier cochlear
implants. That was reinforced and since that time there were no device failures. Thanks.
DR. RICHARD CHOLE.
Are there any other questions?
DR. RICHARD CHOLE.
A question for Dr. Jeffrey Adams.
The left-sided results being better than the right caught my interest and I’m sure many others. Is
that just a selection process or a handedness problem, can you explain?
DR. JEFFREY ADAMS, DAYTON, OH
I don’t know exactly what to make of it. Obviously there was a lot of selection bias in the study.
I don’t know if Dr. Sismanis has a theory on it.
DR. ARISTIDES SISMANIS, RICHMOND, VA
I don’t know that it’s significant, to be honest with you.
DR. CHOLE:
Any other additional questions? We are slightly ahead of time. Could I remind you all to get
your photographs? You have to pick up a number, sign your name next to that same number.
The photographs will be out where the birds are. If you all remember where the parrots and
parakeets are out there, that is where we are going to have the photograph. Also I think we can
go ahead with the next session.
LABYRINTHINE ANOMALIES:
Dehiscence of Bone Overlying the Superior Semicircular Canal as a
Cause of Apparent Conductive Hearing Loss
Lloyd B. Minor, MD, John P. Carey, MD
Phillip D. Cremer, MD, PhD, Sven-Olrik Streubel, MD
Objective: To identify superior canal dehiscence in patients with apparent conductive hearing loss and to define the
cause of the air-bone gap.
Study design: Prospective study of patients with superior canal dehiscence.
Setting: Tertiary referral center. IRB Approval #99-06-29-02.
Patients: Vestibular signs and/or symptoms of superior canal dehiscence and findings on high-resolution CT scans
of the temporal bone.
Interventions: Vestibular-evoked myogenic potential (VEMP responses), three-dimensional eye movement
recordings.
Outcome measure: Association of superior canal dehiscence with air-bone gap and intact VEMP responses.
Results: Three patients with dehiscence of bone overlying the superior canal were noted to have air-bone gaps in
the affected ear measuring 25-40 dB for frequencies of 250 - 2000 Hz. Prior to identification of superior canal
dehiscence, each of these patients had undergone stapedectomy for correction of presumed impairment of stapes
mobility. The air-bone gap was unchanged postoperatively. Each patient had an intact VEMP response from the
affected ear, a finding that would not have been expected based upon a middle ear cause of the conductive hearing
loss. One patient underwent resurfacing of the superior canal through a middle fossa approach. Postoperatively, his
vestibular symptoms were relieved and his air conduction thresholds were improved by 20 dB.
Conclusions: Superior canal dehiscence can result in apparent conductive hearing loss. The 'third mobile window'
created by the dehiscent superior canal results in dissipation of acoustic energy and is a cause of 'inner ear
conductive hearing loss'.
Lloyd B. Minor, M.D.
Dept. of Otolaryngology
Johns Hopkins Outpatient Center
601 N. Caroline St., Rm. 6253
Baltimore, MD 21287-0910
A Novel Stapes Ankylosis Syndrome is Caused by Mutations in the NOG Gene
David J. Brown MD, Theresa B. Kim BA, Elizabeth M. Petty MD,
Catherine A. Downs MS, Donna M. Martin MD PhD, Peter J. Strouse MD
Sayoko E. Moroi MD PhD, Stephen S. Gebarski MD, Marci M. Lesperance MD
Hypothesis: An autosomal dominant syndrome of stapes ankylosis, hyperopia and skeletal abnormalities is caused
by a mutation in a single gene, hypothesized to be the NOG gene.
Background: We studied a family with bilateral congenital stapes ankylosis associated with hyperopia, broad
dysmorphic thumbs and great toes, joint abnormalities, and syndactyly, without symphalangism or features of
osteogenesis imperfecta. Mutations in the NOG gene are known to underlie similar but distinct stapes ankylosis
syndromes.
Methods: DNA sequencing of the NOG gene was performed for 8 affected individuals, 3 unaffected relatives, and
100 controls. Clinical and genetic data were correlated.
Results: A novel NOG mutation, 1139C>T, Q110X, was found in all affected individuals but not in unaffected
family members or controls. The Q110X mutation creates a premature stop codon that truncates the protein from
232 to 110 amino acids.
Conclusions: While the presumptive diagnosis was initially otosclerosis, further investigation identified subtle
skeletal features comprising a congenital stapes ankylosis syndrome. The Q110X mutation causes milder effects
than those reported for other NOG mutations. NOG encodes a secreted protein, noggin, that interacts with bone
morphogenic proteins (BMPs) in the extracellular matrix and is essential for normal bone and joint development.
The mutant protein may disrupt the BMP pathway or it may be degraded, resulting in insufficient amounts of normal
protein. Identifying the genetic cause of a congenital stapes ankylosis syndrome may help identify targets for future
therapeutic intervention.
Supported by the AOS Research Fund and the General Clinical Research Center
#M01-RR00042.
IRB #1996-597
Marci M. Lesperance, M.D.
F6905 Mott
1500 E. Medical Center Dr.
Ann Arbor, MI 48109-0241
Enlarged Vestibular Aqueduct Syndrome
in the Pediatric Population
Colm Madden, MB FRCSI, Corning Benton, MD FACR
John Greinwald, MD, Daniel Choo, MD
Objective: To correlate clinical and audiometric findings with the radiological appearance in patients with enlarged
vestibular aqueducts (EVA).
Design: A retrospective review of a Pediatric hearing loss database of over 820 patients.
Setting: A tertiary Pediatric referral center.
Patients: Subjects were included for study with a radiographic diagnosis of EVA in at least one ear by a
neuroradiologist.
Intervention: N/A.
Outcome Measures: Audiometric performance and vestibular aqueduct width.
Results: Forty-six patients (76 ears) were identified with an EVA with a M:F ratio of nearly 1:1. Patients were
followed for a mean of 46 months (range 2 - 205 months). Hearing loss was bilateral in 65% of cases. Associated
inner ear pathologies were visualized on 25% of the CT scans. Vertigo was present in only 4 (9%) of the patients
and was associated with a sudden drop in hearing after mild head trauma. Borderline enlargement of the vestibular
aqueduct was associated with varying degrees of SNHL. 40% of ears had the characteristic low frequency
conductive or mixed loss seen in this condition, despite a normal middle ear. Overall, the audiogram remained stable
in 57% of ears, fluctuated in 18% and progressively worsened in 25%. Treatment included hearing aids (58%), FM
systems (43%), preferential seating (33%) and cochlear implantation (7%).
Conclusions: Audiometric thresholds remained surprisingly stable. Sudden deterioration of hearing following head
trauma was seen in four male patients, prompting speculation that males are more likely to encounter head trauma
and thus are at higher risk of sudden hearing loss. Cochlear implantation was of significant benefit to those children
selected for implantation.
Colm Madden, M.B., FRCSI
Dept. of Pediatric Otolaryngology
Children's Hospital Medical Center
3333 Burnet Ave.
Cincinnati, OH 45229
Discussion Period Papers 10-12
DR. RICK CHOLE:
This paper will be open for discussion in just a minute but let me remind the members of two
things. One to pick up a little card like this at the desk outside and sign your name. That’s going
to be for the photograph. The photograph will be held immediately after the discussion section
out on the Spanish terrace there which is where the parrots and birds are. So please make it there
promptly and we can get this done quickly. These papers are now open for discussion.
DR, THOMAS VAN DE WATER, MIAMI, FL
I had a question for Dr. Brown about the NOG mutation. As you probably know from work that
was also done at the University of Michigan in birds by Kate Beraldin and Margaret Lomax,
noggin is associated with malformations of the semicircular canal and yet I don’t think you saw
any of that in any of the patients, which is very interesting. What do you think is going on there?
DR. DAVID BROWN, ANN ARBOR, MI
Yes I do know Dr. Beraldin and Dr. Lomax, I worked with them as well. It is true there is no
vertigo, no dizziness in this family and there were no anomalies found on the computer
tomography scans. In those experiments, if I remember correctly, they put beads in the
semicircular canals with and without noggin and they showed that they can inhibit semicircular
canal formation. In this family it seems like there’s a gradient type of thing in which there is a
certain amount of noggin that is available to inhibit the bone morphogenetic proteins. So, it
might be more of a gradient thing.
DR. RICHARD CHOLE
My question is did you say that other noggin mutations have different phenol types and if so
how do you explain that? Is there splice variance with the noggin gene or what is the mechanism
of that?
DR. DAVID BROWN:
Well the NOG gene is a single gene and there are different mutations causing different types of
frame-ships. Ours is one of the two that I know that actually has a stop code on and some others
might just have a change in a base pair but each of them has a slightly different phenotype and
there are a lot of consistencies that are conserved and we are not sure. There are not many
functional studies have been done and were not sure how each of the mutations actually change
the confirmation of the noggin and how that affects the interaction with other proteins so there is
a spectrum with the extreme being more the bony changes which was not present in our family
and hyperopia which is far sighted vision and the reason why we suspect that is because noggin
was first found in the central nervous system development where brain dorsal ventralization and
central nervous system so were not sure how each of the mutations affects the confirmation of
the noggin protein.
DR. JOHN SHEA, MEMPHIS:
I’d like to comment on Dr. Minor’s paper for several reasons, one of which it’s one of the papers
I can sort of understand this afternoon. The rest of the program was wonderful but this business
of this so-called internal conductive hearing loss is an old and big problem. When I was working
with Howard House way back in 1953 and we were doing fenestrations, he was aware of this
syndrome and first called it to my attention and I tried to understand it but there’s one additional
fact Lloyd that you may or may not know and I don’t think I heard you say it. When you open
the middle ear of a person with conductive hearing loss, you of course ought to see if there’s
some otosclerosis present, but you also ought to test whether the round window reflex is present
because in these people with this so-called internal conductive hearing loss that John House
wrote this paper about, they don’t have a round window reflex and that tends to add to your idea.
That is, a loss of the acoustic or hydraulic motion of the fluid in the middle ear. But it’s a
fascinating group of patients and maybe this superior semicircular canal defect is the cause. I
must say I have never seen a patient with this syndrome but you have seen thirteen of them so I
must have missed some of them. Thank you.
DR. LLOYD MINOR, BALTIMORE, MD
Just a brief comment to Dr. Shea and thank you very much. The other entity to keep in mind is
enlarged vestibular aqueduct syndrome that Dr. Madden so eloquently described. There was a
paper in American Journal of Otology two years ago describing abnormal vestibular myogenic
potentials in patients with enlarged vestibular aqueduct syndrome and the pattern of vamp
abnormality is similar to that in superior canal dehiscence and it makes sense because enlarged
vestibular aqueduct syndrome can act as well as the third mobile window so that may also
account for some of the cases of inner ear conductive hearing loss. Of course the temporal bone
CT scan these days will identify either problem.
DR. SAUMIL MERCHANT, BOSTON, MA,
Lloyd I enjoyed your paper and I have a question for you. At our place we have seen three our
four patients with the exact same clinical presentation that you described. A relatively large air
bone gap, no abnormality in the middle ear when you explore them and a dehiscent canal and
some have a vestibular problems and some do not. But one thing we have noticed is that the
bone conduction thresholds in all of the individuals that we have seen with this problem are
better than normal in the lower frequencies minus five minus ten and we think that John who is
here with me, one of our auditory scientist, we think it may be that bone conduction thresholds
are slightly improved because of the abnormal mechanics due to the dehiscence. My question to
you is have you noticed that in your patients? The other comment is that when audiologists do
audiograms, when they reach a bone threshold of zero, they just stop and they don’t find the
absolute bone threshold. I think we need to tell our audiologists to look for this problem because
I think the bone conduction audiometry can be a clue to diagnosing this and not exploring these
patients unnecessarily.
DR. LLOYD MINOR,
Yes we have also seen that when we use an audiometry calibrated down to minus ten minus
fifteen DBHL that often times the bone conduction thresholds are supra normal and that fits also
with the fact that I think in every patient I’ve seen with superior canal dehiscence syndrome the
Weber has lateralized to the affected ear even when the ear conduction thresholds are completely
in the normal range. So there is a conductive hyperacusis I think due to this canal dehiscence. In
the patients that I talked about today, three of them had prior stapes surgery so that point in the
bone conduction thresholds may be more difficult to interpret but in the group of superior canal
dehiscence canal patients in general that’s been a common feature is that the bone conduction
features are better than zero DBHL.
DR. CHARLES LUETJE, KANSAS CITY, MO
Lloyd in the absence of any ossicular change fixation and you saw pulsation of the round
window membrane window on exploration synchronous with a heartbeat, would you expect
there to be any problems with the semicircular canal or dehiscence?
DR. LLOYD MINOR:
So this is someone who had an air bone gap on the audiogram preop and you are exploring the
ear and the stapes moves fine and the chain looks fine but you see the round window membrane
pulsating?
DR. CHARLES LUETJE, KANSAS CITY, MO,
Yes, correct.
DR. MINOR:
I think that would be a suspicion that there is another mobile window someplace leading to that
pulsation.
DR. JJIM SAUNDERS, OKLAHOMA CITY, OK
I have a question for Dr. Madden. I was curious by your numbers for progressive hearing loss
and also the association with head trauma. I was wondering how that compares with your overall
group of 1200 children with sensorineural hearing loss. Are those significantly different from
the group at large?
DR. COLM MADDEN, CINCINNATI, OH
The group with progressive loss was smaller than has been reported in other studies. We have a
reduced number of people who have come to us with a history of sudden sensorineural hearing
loss following head trauma. Most studies have shown much larger numbers. Perhaps one of
those reasons is the now common observation that is passed on to the parents of these children to
avoid head trauma. Interestingly enough in the three children that had dips in their hearing
following head trauma, all were male, which may predispose males more than females to head
trauma in that pediatric age group.
DR. JIM SAUNDERS, OKLAHOMA CITY, OK
So you haven’t actually looked at the larger group to see whether there were any in that group
that had dips with head trauma? In a sensorineural group at large, in other words not the
enlargements to their aqueduct?
DR. COLM MADDEN, CINCINNATI, OH
No, I haven’t analyzed the whole sensorineural group.
DR. JIM SAUNDERS, OKLAHOMA CITY, OK
And was there any difference in those associated with known syndromes in the Pendrods or the
BOR’s? Did you notice any difference in those?
DR. COLM MADDEN, CINCINNATI, OH
No not specifically no. Thank you.
DR. CHOLE:
Any further questions? We have another announcement for the members and guests. The
banquet location for tomorrow night has been changed to the Estate Ballroom in the Meisner
Center. So we will announce that again tomorrow. This session is now closed. Thank you and
we will see you tomorrow.
.
SCIENTIFIC PROGRAM
Saturday, May 11, 2002
Cochlea
Presumptive Cochlear Hemorrhage as the Source of
Sudden Sensorineural Hearing Loss in Five Patients
Mark D. Packer, MD, Brian P. Perry, MD
Clinical Study
Objective: To propose cochlear hemorrhage as an identifiable mechanism of sudden
sensorineural hearing loss.
Study Design: Case series
Background:
Five patients with sudden sensorineural hearing loss noted to have
intracochlear hemorrhage by MRI.
Setting: Tertiary care hospital
Results: Five patients with sudden sensorineural hearing loss attributed to cochlear
hemorrhage.
Conclusion: Cochlear hemorrhage can account for some cases of sudden sensorineural
hearing loss, may be related to coumadin treatment, and can be identified by MRI.
Routine MR imaging for sudden sensorineural hearing loss (SSNHL) has been shown to increase the diagnostic
success for this commonly “idiopathic” disease. It will identify the 10% of vestibular schwannoma cases, and the
5% of multiple sclerosis cases that present with SSNHL. It can also help document ischemia, infectious
labyrinthitis, vascular malformations and several other disease processes. Presented here are five cases of SSNHL,
two bilateral, with MR sequences suggestive of intracochlear hemorrhage by their pre-contrast, high T1 signal
intensity. The high signal intensity corresponds to the increase in methemaglobin levels of subacute and chronic
bleeds. Three of these patients were taking coumadin, and one had Ehrler-Danlos syndrome. Although treated with
steroids and antiviral medications, only one patient showed significant improvement in hearing post treatment.
Cochlear hemorrhage should be considered when evaluating patients with sudden sensorineural hearing loss when
precontrast T1 images by MR show high signal intensity.
Mark D. Packer, M.D.
8414 Blackcastle Dr.
San Antonio, TX 78250
Etanercept-Suppressed Inflammation in Mouse KLH Induced Labyrinthitis
Hitoshi Satoh, MD, PhD, Elizabeth M. Keithley, PhD, Gary S. Firestein, MD
Peter Billings, PhD, Jeffrey P. Harris, MD, PhD
Hypothesis: TNF-a plays an important role in initiation of cochlear secondary immune response, and a recombinant
human TNF receptor Fc fusion protein (Etanercept) can reduce the inflammation.
Background: The inner ear is capable of rapidly mounting an immune response that can ultimately lead to cochlear
degeneration and permanent hearing loss. Blocking the response should prevent these damaging sequelae. The
identification of preinflammatory cytokines (IL-1, IL-6 and TNF-a) within the inner ear should lead to rational
therapeutic strategies.
Methods and Results: A secondary inner ear immune response to keyhole limpet hemocyanin was created in the
mouse cochlea. The animals were sacrificed 3-48 hrs and 7 days following initiation of the immune response. The
cochleas were assayed for the expression of the preinflammatory cytokines, IL-1b, TNF-a and IL-6, by
immunocytochemistry. IL-1b and TNF-a were expressed by infiltrated cells shortly after KLH injection. IL-1b was
also expressed by the type I fibrocytes of the spiral ligament. IL-6 is expressed in normal cochleas, but did not show
much change during the immune response. Surgical controls showed IL-1b, but not TNF-a, and intrathecal
injection, which can eliminate the surgical effect, showed TNF-a expression. Additionally, systemic injection of
Etanercept reduced cochlear infiltrating cell number and cochlear fibrosis evaluated 7 days after KLH injection.
Conclusions: It is concluded that IL-1b expression is a general cochlear response to trauma and that TNF-a is
responsible for amplification of the secondary immune response that leads to pathology. Treatment by Etanercept
can reduce the cochlear damaging sequelae induced by the cochlear immune response.
Jeffrey P. Harris, M.D., Ph.D.
200 W. Arbor Drive, #8895
San Diego, CA 92103
Discussion Period 4 Papers 13-15
DR. AZIZ BEAL, ALEXANDER EGYPT:
I would like to comment on Dr. Packer’s paper. I feel that persistent high signal that was seen
four years after the cochlear hemorrhage was not due to the hemorrhage itself but rather to a new
bone formation inside the cochlea. We feel the same, that is not persistence of clot or
hemorrhage necessarily to account for the persistent high signal but their feeling is that it is more
consistent with protein presenting sclerosis within the cochlear labyrinth itself. It would be
interesting to have the CT imaging on these patients and that may show if there’s further new
bone formation.
DR. MARK PACKER, SAN ANTONIO, TX,
Yes. In discussion with our neuroradiologist DR. CHOLE:
As I mentioned the second paper has been withdrawn. The next paper for presentation is by
Hitoshi Sato and colleagues - Eternocept suppressed inflammation in mouse KLH induced
Labryinthitis..
DR. JACK PULEC, LOS ANGELES,
My question is directed to Dr. Satoh. It seemed a little ironic to be speaking about etanercept
after the loss of our member, Jean- Bernard Causse. Would you explain or tell us the source of
the Enteracept.
DR. HITOSHI SATOH, SAN DIEGO, CA
Etanercept antibody is all in the system in Minneapolis, is that okay?
We bought the antibody from the LMD systems in Minneapolis, USA.
DR. RICHARD CHOLE
I had a question for Dr. Packer. If I understand correctly the evidence you presented that it’s a
hemorrhage, is that the T1 signal was low the first day and then became high in the days after
that. Is that correct, is that the evidence for it actually being hemorrhage?
DR. MARK PACKER, SAN ANTONIO, TX
that’s part of the evidence. We had two patients that initial negative MR imaging and that
follows the progression as shown with intracerebral hemorrhaging. Further evidence we don’t
have direct definitive knowledge that this is hemorrhage but the imaging is consistent.. With the
past research the things that account for the high T1 signal there have been retrosigmoid
resections of acoustics and the postop hemorrhaging shows hyperintensity of the T1 signal.
Other conditions that show high protein have not also shown the high T1 signal.
DR. ROBERT CEUVA, SAN DIEGO, CA,
Also for Dr. Packer, I was going to address that same issue as Dr. Chole. In my experience
fortunately in working with neurosurgeons over several years, I have seen a lot of patients with
intracranial hemorrhage. We have a routine neuroradiology conference at the institution at
which I work and it has been my impression that the delay, the methemoglobin is much brighter
than the images that you portrayed. The cochlea looked pretty much isointense with brain where
his methemoglobin is very bright on T1 on contrast almost to the intensity of fat. Did your
neuroradiologist do measurements in terms of the signal intensity of the specific areas and
correlate them with other patients with known hemorrhage? That might be a way to help you to
see is that hemorrhage or does it match up with another tissue type?
DR. MARK PACKER, SAN DIEGO, CA,
Interesting thought. Yes, we did not do those specific imaging techniques however they have
theorized that the difference between the inter-cochlear oxygen content and the more anaerobic
environment of brain bleeds may account for some of that difference.
Acoustic Neuroma Panel
DR. RICHARD CHOLE,
I’d like to invite the panel members to come on to the podium. I have asked a few neurotologists
who are active in acoustic neuroma treatment in surgery to respond to some treatment questions
about acoustic neuroma. I was thinking about this and this is a panel we probably could have put
on every Otological Society since the treatment parameters and management parameters for
acoustic neuromas seem to be changing every year. The panelists are Joel Goebel who is a
Professor at Washington University, Bruce Gantz, Department Head at Iowa, John Niparko
Professor at Johns Hopkins and Rick Friedman from the House Ear Institute. Phil Wackym
unfortunately couldn’t be here. Phil was playing soccer with his eight year old, fell down and
broke his clavicle in four places and several ribs so we are currently screening him for
osteogenesis
imperfecta but he had open surgery on his clavicle and couldn’t be here. Phil is doing gamma
knife treatment of acoustic neuroma so we are going to miss him on this panel but I’m sure that
the rest of our panelist will represent him very well. I chose to present a few cases and we are
going to have some kind of open discussion about management of these cases.
I will just get started.
The first case is a 12-year old girl who really has had no symptoms. She and her grandmother
have neurofibromatosis, her mother recently diagnosed. Her mother has hearing loss in one ear
and her grandmother is completely deaf due to eighth nerve tumors. So in her evaluation,
audiograms were done and her hearing is really perfectly normal. I’m hoping the panelists are
going to be able to see the films okay, they were given to them earlier. This is her MRI. This is
an enhanced scan with gadolinium and you can see small tumors in both internal and auditory
canals. So we now have a 12-year-old girl pretty well documented with neurofibromatosis II and
the parents and child are asking for treatment and recommendations. I will give it to the panel
and ask for comments. Maybe I could start with Rick.
DR. RICK FRIEDMAN:
This is obviously a difficult problem with a young girl. We have just recently published our
series in our philosophy on the management of NF2. Obviously all of the options would be
discussed with this family and child. The options would include as we al know observation,
radiotherapy or microsugery. It’s been our philosophy to manage these patients early when
tumors are small and function is good. So in a case like this my recommendation, assuming the
child was healthy would be middle fossa removal of the smallest tumor on the right since the
hearing is equivalent, maybe some more data like ABR or ENG. Although we did look at ENG
and it didn’t really in our series affect hearing outcome. I may look at ABR because these are
both relatively small tumors and maybe select the side that was causing less of a problem with
the auditory nerve. But in general we would manage this I believe with a middle fossa.
PANEL MEMBER NOT IDENTIFIED:
I think that is right given the lateral location of these tumors, a retrosigmoid approach which is
more commonly used in our institution, would not be in this little girl’s best interest. As we all
know these are difficult cases particularly with the relatively few symptoms experienced by the
child and there may be a great resistance on the part of the child themselves and extensive
counseling is needed for these cases. I would add backing up what Rick said about early
intervention. In fact we have had similar size lesions in NF2 patients that turned out to be facial
neuromas and in two cases were able to excise those neuromas with nearly normal facial
function postoperatively. It’s a rare manifestation of NF2 but if either of these signals represents
a facial neuroma, she’s best served with early intervention.
PANEL MEMBER NOT IDENTIFIED:
Well I have had this situation in a set of twins at fourteen years of age and I can tell you that we
did remove the smaller tumor in both children. In one child we were able to save the hearing and
the person is now in college and doing well. His brother, we were not able to save the hearing
and then the larger tumor continued to grow and in June we are going to have to take out that
tumor and put in an ABI. These are extremely difficult cases but if you can have a winner and I
have six patients that I have operated on with the smaller tumor and have been able to save
hearing in four but it’s a crapshoot. You are not guaranteed that you are going to be able to save
the hearing but this is the best chance when it’s small.
PANEL MEMBER NOT IDENTIFIED:
Yeah I think that makes sense that it almost has a parallel in stapes surgery when you talk to
patients about bilateral otosclerosis. These tumors can do individually what they want. They
can have individual growth rates and like Bruce said if you hit a home run on one side you are in
a good position and if you don’t then you are in a real sticky wicket so I would agree with going
after it aggressively because you have the best chance of managing this when the tumors are
small.
PANEL MEMBER NOT IDENTIFIED:
I know Bill Slattery presented it last year and that is for the unfortunate case where you loose
hearing despite early intervention, middle fossa decompression has really shown to give fairly
long-term in many of the patients reviewed, hearing preservation in the only hearing ear with the
tumor remaining so that’s also something included and discussed thoroughly with the patients
and family.
DR. CHOLE:
No one is suggesting watchful waiting or radiation?
PANEL MEMBER NOT IDENTIFIED:
I think she’s much too young for interval scanning. I don’t think that is a wise approach at all. I
do want to say having recently dealt with two similar cases, there are radiation therapists in our
region that would radiate in such cases and there are some who are very steadfastly opposed to
radiation in these cases. I think its incumbent on us to get to know our radiation therapists as
best as you can. I would submit to this audience that there’s a lot of internet play that is really
quite disingenuous when it comes to radiation of a benign tumor and we I think are placed in the
position of sorting that out for our patients.
DR. CHOLE:
Okay, let me go to the next case. This is a case of a 54-year-old healthy audiology researcher
actually who noticed tinnitus in his left ear in the preceding few months. He did an audiogram
on himself and found a mild high frequency hearing loss in one ear. Excellent word
discrimination score but was worried about this and an MRI scan was performed. This is his
scan. You can see that there’s a small enhancing lesion in the mid -part of the internal auditory
canal. So 54-years-old, healthy and having very few symptoms. Let me start with John this
time.
DR. JOHN NIPARKO:
Quite lateral signal. I think the differential has to be opened up on this a little bit and you do
have to make sure you aren’t looking at an inflammatory lesion there that could be related to
sarcoid or lyme disease or something like that. Assuming Rick that you are not trying to trick us
at all and that we have follow up and this is indeed a schwannoma in a 54-year old gentleman,
he’s going to be very tempted to have this radiated. I personally would believe that if he’s got
relatively few symptoms at this point and a very mild hearing loss that I would probably coach
him to consider an interval scan in three to six months, careful audiometry as well. The risk of
that of course is that you may loose your option for hearing preservation.
PANEL MEMBER NOT IDENTIFIED:
Well if this person came to you and was concerned and you found this lesion you have to discuss
it with the patient and find out what the patient would like to do. Some people are interested in
interval scans but someone like this is probably not if he’s interested in trying to preserve his
hearing and I would pursue middle fossa with this person if they were interested in surgical
excision.
DR. GOEBEL:
The hallmark of the radiotherapy literature is not the disappearance of the lesion but the arrest of
the lesion and you would never know if he chose radiotherapy for this lesion because its small
and you have no idea of growth at all. I think radiotherapy would not be an issue at this point. I
think you would be between what Bruce said as either your best chance of taking this out and
preserving hearing versus an interval scan and that would depend on the mind set of the patient.
These are all very savvy people. They know about serial observation MR, they know about
radiotherapy and they know about surgery so you really would have to dig into his head, no pun
intended to find out between observation and taking the tumor out
DR. CHOLE:
Let me get down to the actuality of this here. He’s sitting in your office and your talking to him
about these options and certainly a case like this you might consider all three options. Would
you send him to the radiotherapist for consultation and opinion or right then and there
recommend treatment? Joel?
DR. GOEBEL:
Every institution has their availability of radiotherapy, either its right there set up or its not. In
Washington University every one of our patients if they are sent for the neurosurgical opinion
they are automatically sent for the radiotherapy opinion, it’s coupled. I think even though you
wouldn’t necessarily recommend it as his treating physician in this case, I think you are
behooved for them to hear the argument. The important point is that they come back to touch
base to you so that you can wrap it up for them. You have to emphasize to them you are their
treating physician; you are going to let them hear the options. So in our institution if you
involved neurosurgery at all they would automatically be sent for an opinion.
DR. CHOLE:
That will get some blood pressure up, let me get some opinions. Go ahead John.
DR. NIPARKO:
Rick you have submitted to us this sophisticated individual and I would submit to you that a
sophisticated individual would want to look at the quality of the evidence and the literature. I
would call your attention to a recent publication from the British Society’s of Neurosurgery and
Otolaryngology that did an exhaustive evaluation of the literature on radiation therapy for
acoustic tumors. In fact they rated the publications from Grade 1 which is the highest level of
prospective trial dated down to Grade 4 which is basically inadequately assessed case series and
conflicting evidence. The vast majority of publications in fact are Grade 4 in terms of their
quality. There are few Grade 3, no Grades1 or 2 publications on the Affect of Radiation Therapy
for AcousticTumors. It’s really quite impressive. Rob Jackler, John McElveen, Bruce Gantz and
others have reviewed that document. It’s a white sheet that I think is going to be quite important
for us to distribute for our patients.
DR. CHOLE:
You have a comment Rick?
DR. FRIEDMAN:
I think I agree with every single comment here at the table. I mean a person like this obvious is
individualized. A 54-year-old is probably going to have to deal with this at some point in his or
her life and the philosophy I think for most of us and certainly at the House Ear Clinic has
always been to intervene early, to enhance your postoperative outcomes. So I agree with Bruce.
This person is probably going to want to save hearing and I think you have to educate a lot of
these patients. You may not have one as sophisticated as this. The radiosurgical literature really
focuses on preservation of function but the philosophy and treatment is for cure. If you’re going
to treat a tumor, your treating it so that the tumor is over and there is no long-term data to show
that. If you look back to what John was saying about the quality of publications…if you look at
the Pittsburgh series, I think it was in 2000 or 2001 that Flickinger reported on their five year
actuarial control which is even of itself I think an oxymoron. I’m not sure you can look at
actuarial control in benign disease but anyway if you look closely at the fine print, there was 30
months of median follow up. At the 30 month mark there had already been 3-5% of patients
who required surgery and if you looked at the other criteria they were describing for failure
which they downplay on their discussion and that was radiographic evidence of change. If you
look at the arrow bars it could be as high as 15% at thirty months. Those are orders of
magnitude greater than most microsurgical series so I think the patients really have to understand
that the issue is not saving your hearing, the issue is doing this one time and being finished with
it and doing it the best way you can to preserve function.
DR. CHOLE:
Next case is an interesting case of a 15-year-old boy who presented with episodes of vertigo,
sudden onset and lasted about two hours, very Meniere’s like. They occurred about once a
month and he recently noticed some hearing loss in his left ear. This is his audiogram, which
shows normal hearing in the right ear but a mixed hearing loss in his left ear. Because of the
mixed hearing loss and discrimination score of 76%, he had an MRI scan. This scan was read as
normal by the University neuroradiologists and I would like to point this out for any of the
residents or younger members in the audience except radiologists reports. There’s probably not
a person in the room that accepts a radiologists report but this was the report by a qualified
neuroradiologist. Actually, embarrassingly, I had written a paper with this man once about a
similar issue and this was the scan. Could I ask the individuals on the panel to take a look at the
scan. I sent them a power point of this before so you would have a little bit of a chance to look at
it. It’s kind of hard to see from here. Let me start with Rick
.
DR. FRIEDMAN:
I don’t want to embarrass myself. I didn’t stare at the power point. If I’m seeing it correctly,
does this look like an intra-labyrinthine neuroma? Seeing it from this angle?
DR. CHOLE:
This is an enhanced scan, gadolinium scan with a signal in the labyrinth.
DR. FRIEDMAN:
I just saw one of these last week. It’s a complicated issue obviously, there’s no such thing as
preservation of hearing in cases like this
DR. CHOLE:
What about the audiogram in comparison to this? How would you compare his audiogram?
DR. FRIEDMAN:
That’s a good question. Obviously the high frequency sensorineural loss is from the
schwannoma in the vestibule and in the cochlea. The conductive component, there’s a mass
affect in the inner ear that could be “a cochlear conductive phenomenon”. I don’t see anything
protruding out of the labyrinth into the middle ear. I have seen that once actually a schwannoma
in the middle ear. Obviously that patient had no hearing it had blown through the footplate of
the stapes but I would guess this is similar to the X-linked deafness, mixed deafness patients who
have rather than tumors CSF pressure coming through an absent modiolis causing again “a
cochlear conductive loss” so I guess its probably the mass affect assuming no other middle ear
disease.
DR. CHOLE:
Let’s ask the panelists about diagnosis here. I may have another scan here to clarify this just a
little bit. This is a T-2 scan and if you look carefully here you can see right in the vestibule a
negative image right here in the signal of the fluid compared to the other side so you can see a
mass affect right in the vestibule there. This is the magnified view of the T-2. Other panelists,
comments about it, Joel?
DR. GOEBEL:
I had a patient and have a colleague in the audience who helped with this because he had
bounced around a bit that really carried a diagnosis of Meniere’s disease. He had all of the
characteristics and he had a positive ECOG and negative ABR . He had attacks and it responded
to diuretic and low salt diet and looked just like Meniere’s and he turned out eventually when he
was scanned to have a very tiny intra-labyrinthine schwannoma so it was almost exactly this
picture. Your right, you cant’ save hearing on an issue like this, so it’s not a matter of offering
him a surgical approach to save hearing. In an instance like this I would probably watch him
until his hearing dropped or the lesion looked like it was growing and then you’d have to deal
with it at that point.
DR. FRIEDMAN:
I agree with that and we have had this situation and some of them don’t grow. You watch them
and rescan them on a yearly basis. I think I had one that did grow and it destroyed the hearing
and it was growing so we decided to do a labyrinthectomy and take care of the problem because
we thought we would put the facial nerve at risk over a long period of time so that’s why we
removed the tumor. But you are not going to save hearing and watchful waiting is probably the
best approach for a patient like this and just explain to them there’s nothing you can do to stop
that.
PANEL MEMBER NOT IDENTIFIED:
Yes Rick just mentioned that the problem is the symptoms and the abruptness of the symptoms.
My experience in just a couple of these cases is that in fact they do have very severe symptoms
that can come on without warning so if they do a lot of driving, if they are aviators that sort of
thing this can be a greater risk for them.
DR. CHOLE:
He has had some vertigo spells but they have not really worsened and the option I pretty much
gave him we were watching for the moment. The thing that is a little disturbing to me is that
he’s 15-years-old and I’m diagnosing a schwannoma on him. Are there other possibilities that I
should maybe worry about, any chance of malignancy or anything that needs more urgent
treatment of a tumor like this?
PANEL MEMBER NOT IDENTIFIED:
The obvious risk for this child is the subsequent development of NF2 or the existence of NF2 but
its manifestations in the opposite nerves or other cranial nerves. So I think it’s obvious that the
child needs follow up for a fair number of years to observe the opposite side to observe the other
cranial nerves at the skull base. As far as malignancy goes with the severity of the symptoms for
such a small size lesion, I don’t believe so.
DR. CHOLE:
Let me ask you another question. Would you screen this child genetically for NF2?
It’s an expensive proposition usually not covered by insurance but it’s available?
PANEL MEMBER NOT IDENTIFIED:
I’ll comment to that. I personally don’t think I would. I think we’ve got a good diagnostic
modality with MR and there are a number of false negatives in NF2 screening. I see Brad here
so I don’t want him to kick me for saying anything wrong but I guess a lot of NF2 tumors show
no mutation and coding regions of the NF2 gene. A negative screen certainly doesn’t mean the
child is out of the woods. I think MR would be the best way to go.
DR. CHOLE:
The parents are very sophisticated and they did do NF2 testing which was negative and then
understood the problems with it.
The next case is a 28-year-old healthy man who has noticed hearing loss and ringing in his left
ear over the past several months. He’s had no imbalance or vertigo. He’s a professional person
who works with the public and is very concerned about his hearing and balance. This is his
audiogram showing a mild to moderate high frequency hearing loss, word discrimination score
on the affected ear is 84% and here is the scan of his tumor medium size coming out of the
internal auditory canal into the cerebellar pontine angle. What do you do? We will start this
time with Bruce.
DR. GANTZ:
This is more of a medial placed tumor. People that do a lot of retrosigmoid tumor removal
would most likely approach it from that perspective. In my hands when these tumors touch the
brainstem like that the chances of saving hearing are not really great. I mean you can’t say that
in this tumor you have a 50% chance of saving hearing. I think it drops quite significantly. It’s a
little big for a middle fossa but we have done some middle fossa’s on tumors that size and I
guess I would talk to him about those kinds of things. I’ve had a situation where I’ve done a
retrosigmoid and the MRI scan didn’t tell me the truth because there was tumor going way out
lateral. So I think you have to be concerned about that and if you have a good neurosurgeon that
you can work with and do a retrosigmoid, that might be the best approach. I would probably do
a combination of a retrosigmoid retrolab and see if I could get it out. If I couldn’t I would just do
a translab.
DR. CHOLE:
Joel, could you comment. Since this is actually your case let me have you comment on that.
DR. GOEBEL:
This has a lot of confounding factors because of who he is. Rick said he’s interested in his
hearing and balance; he’s very interested in his face. Even a Grade 2 facial weakness to this
fellow who is a young strapping looking stockbroker that is the part that really pulled him back.
Even the outside chance of facial weakness just absolutely floored him. So we had this
discussion with him about all three modalities and told him observation is probably not smart.
So his options are to have this tumor removed which we could not deny the chance of temporary
weakness and the possibility of mild weakness with the outside possibility of even worse. He
was sent to my neurosurgical colleague who evaluated him and went through the same surgical
spiel but like I said sent him also to the gamma knife which is run by one of the neurosurgeons in
conjunction with the radiotherapist. Do you want me to go to the end of the story?
DR. CHOLE:
Well let me ask about that possibility. We went through this discussion. He’s very anti-surgery
oriented. What are the issues about doing radiation in a 28-year-old, what are some of the issues
about that?
RICK FRIEDMAN:
I put up some slides at Rick’s suggestion. I presented it at last year’s Academy. Unfortunately
or fortunately for those involved in microsurgery the numbers keep climbing for the number of
radiation failures that we have treated at the House Clinic. It’s now 30 and I think with
somebody like this you are going to have to treat them with kid gloves and absolutely honor their
concerns. If you look very closely at the literature for gamma knife for fractionated stereotactic
radiosurgery and microsurgery for tumors of this size which is going to be very difficult for
anybody to do because it’s not presented that way, the risk of facial nerve paralysis is not very
different. I think the patients have to understand that. There may be a slightly greater risk of
facial paresis with the current dosing schemes compared to microsurgery transient facial paresis
or maybe even a grade 2 but facial paralysis for a tumor this size in well trained hands the data is
very similar. So I think this 28-year-old has to understand the unknown and that is the risk of
long-term failure. Again I can show you a few of those slides. I would just explain thoroughly
understanding the patients concerns but explain thoroughly that radiotherapy despite what may
be being said is an unknown in the long term and for a 28-year-old it’s potentially dangerous.
The outcomes after gamma knife for fractionated for microsurgery, the facial nerve outcomes are
not good.
DR. NIPARKO:
I would agree. I think the unknown long-term particularly for a patient this young is the density
of arachnoid adhesions and cerebellar pontine angle post radiation. We agree that you are likely
to have a good facial nerve result even after radiation therapy but the odds of that go way down
in our experience from about a 10% chance of long-term disability to something exceeding 3040%. I would also like to add in our personal experience that at Hopkins we don’t have 30
radiation failures, we now have seven but a
year ago we had zero. I would submit that we are probably seeing the dawn of a new era for
acoustic neuroma surgery.
DR.GANTZ:
I would just comment that we have not seen any radiation failures yet because we don’t do a lot
of radiation in our area so we are fortunate, but I think that’s the issue that you have to really
impress upon this person that if they are worried about their facial nerve, then you have to go to
some person or group that has lots of experience and whether you do it retrosig or translab or
middle fossa, it depends on the experience of the team more than it does the approach. I just
don’t think the hearing is that important to this guy as the facial nerve so if it's the most
important then you would probably do a translab.
DR. CHOLE:
Let’s look at the next case. You’ll see some similarities here.
The patient is a 62-year-old diabetic man who has noticed ringing in his left ear with mild
hearing loss. He has chronic imbalance he has noticed since recovering from a myocardial
infarction two years previously, so kind of a different type of person. Here’s a rather severe
hearing loss with no word discrimination on the affected side and surprisingly his scan looks
very similar to the last one. So the same tumor, how would you treat this tumor? Let me start
with Bruce.
DR. GANTZ:
Well again you have a lot of issues medically on this patient, diabetic. You can give that patient
the option of watching and waiting. It is relatively small, it’s just touching the brainstem and
you can see if it’s going to grow over the next months. If it doesn’t then you might want to go
another year but it most likely will grow and if it does grow then a translabyrinthine approach
would be in my hands the best approach for this patient
DR. GOEBEL:
I have five or six watchful waiting sick people just like this and of the five or six that I have none
of them to date have grown and I have one guy who is eight years out. He’s very ill. He’s a
great surgical risk and he’s also a risk even though these fields are collimated so well. He still
has a dose to surrounding tissues including some of the temporal bone and if developed a deep
skull base osteitis after radiation he would absolutely be a mess to take care of. So if I had him
under those parameters, I would say sir I’ll see you back in six months and we will rescan you
and we will only treat you and operate on you if our hand is forced.
DR. FRIEDMAN:
This is another interesting case and I guess to show my open mindedness again this is a patient
that needs education because certainly in our community we have seen that patients will be
gamma knifed on one scan. I had a 91-year-old gentleman who presented with ear pain. An
otolaryngologist in the community got an MR because he also had an asymmetric hearing loss
and there was an eight millimeter tumor that was immediately gamma knifed and the poor man is
devastatingly dizzy and didn’t respond to a labyrinthectomy. So first of all this patient has to be
informed that given the option for gamma knife immediately without a follow up scan would be
a mistake as well. But I do think that based a lot on the data in the literature it’s not going to
grow and based on the gentleman’s health observation and then potentially gamma knife may be
all that he would need in his life time. I think I feel reasonably comfortable that gamma knife
might hold back most tumors for 5-10 years so I certainly put that into my equation when I have
an elderly patient and infirm patient who I don’t think is going to out-live the potential gamma
knife failure.
DR.CHOLE:
Let me ask you a specific question. Our time is getting short here, maybe more pertinent to the
younger patients than the older. What are the long-term risks of malignancy due to radiation
either fractionated stereotactic radiation or gamma knife in acoustic neuromas? What is your
experience or opinions?
PANEL MEMBER NOT IDENTIFIED:
Well I’d like to comment that we have looked at this on very careful literature search. There are
some legends out there that there are pockets across the world where there are dozens of patients
who have developed malignancy after gamma knife, fractionated stereotactic radiation. I think
in many cases those are very poorly documented and when you actually call the individuals who
made comments at international meetings you can’t get good back up data. On the other hand
there are at least eight well- documented cases where this has occurred. They all fit criteria for
malignant degeneration in the radiation field. I guess the most troubling aspect of those cases
that have been reported is that they have occurred anywhere between 5 and 8 years after
radiation therapy. If you go back to the experience and other body sites where radiation therapy
has been used for benign disease, in fact that latency period appears to be at least 12-15 years.
There may be something about the way radiation therapy has been used at least in the first half of
the 90’s that may increase the risk somewhat. I think that is a significant concern for the longterm that is starting to manifest itself earlier than it would have been predicted.
DR. CHOLE:
Going along with that comment, do you inform your patients as you are referring them for
consultation of the malignancy risk?
PANEL MEMBER NOT IDENTIFIED:
To answer your question , yes I think when you get into these debates with the radiotherapist
they say we overplay that and I certainly don’t think it’s a good idea to overplay it, I mean eight
cases with the number that have been treated isn’t huge but I think something John said is very
true. There’s an article in the 80’s by Breen Flickinger’s and it was an article looking at
stereotactic radiosurgeyr for another benign lesion at the skull base and that’s pituitary adenoma.
It took them 20-30 years of follow up to see. I think it was 37 and almost 50% failure, but the
key to that and if you speak to most radiotherapist and as John said, if you look at the literature at
all sites and included in this paper they had a 3% incidence of malignancy in the field . I
encourage you all to look at it because I think it’s a valuable paper for patients to know about
and so I don’t think you need to over emphasize the risk but I think you certainly need to
emphasize that there is a risk of carcinoma. If you look at the literature also, Derrald Brackmann
brings this point up and it’s a great one, that the mortality rate for microsurgery versus
radiosurgery is no different. There’s an equivalent mortality rate if you look at the literature.
DR. CHOLE:
Other comments about this issue. Joel?
DR. GOEBEL:
I think you also need to be well informed on the radiosurgical literature to tell the difference
between stereotactic surgical approaches that are done in 2002 and radiosurgical approaches or
radiotherapy which seems to get lumped all together. So you need to know if you are going to
make an argument to the patient because they will be presented to them by the radiotherapist that
well we do things differently in 2002 and all this data is based on our techniques going back in
the 80’s and 90’s. So you have to know that data in order to combat it.
PANEL MEMBER NOT IDENTIFIED:
I was just going to quickly say that most likely the larger problem is going to be regrowth
because the radiotherapists have changed their dosage. They are not using as much radiation at
this point as they were ten years ago because of the problems they were having and now when
you have the regrowth you are seeing the problems that the House group and John has had in
removing them with the facial nerve. You tell the patient that and I think that’s a bigger issue to
them than the malignancy.
PANEL MEMBER NOT IDENTIFIED:
One last comment is the biology of radiotherapy and this has been done totally empirically.
There’s no science behind it and if you speak to them at great length which I have about what
they think the radiotherapeutic affects are there’s no doubt that there’s initial cell death to some
of the tumor cells. The rest of it is based on DNA damage and so the cells have two choices.
They either go into apthtotic pathway, they die or they remain in what’s called cell cycle arrest
and anybody who has seen a post gamma knife MR knows that there’s plenty of those cells that
are alive and in “cell cycle arrest” because there’s an enhancing lesion. It’s not scar tissue, it’s
the tumor enhancing. Those cells have two choices from now until the patient dies, either to die
or to go on dividing, they do not remain in indefinite cell cycle arrest to my knowledge and I
have a background in molecular biology not in cell cycle but the people at the institute that are in
cell cycle say there’s no such thing as permanent cell cycle arrest. So they either mutate another
gene that allows them to continue growing or in these eight reported cases the mutated gene
again that allows them to grow out of control. So it’s something that’s going to take a number of
years and decades to resolve.
DR. CHOLE:
I’d like to thank the panelist on some very good remarks, we appreciate it.
Thank you.
Middle Ear/ Mastoid
The Artificial Tympanic Membrane (1840-1910)
From Brilliant Innovation to Quack Device
Eugene A. Chu, BS, Robert K. Jackler, MD
Objective: To utilize the rich and checkered history of the artificial eardrum, a widely utilized device in the 19 th
century, to illustrate the behavior of otologists in response to the introduction of a promising new technology.
Data Sources: Over 40 published books and articles spanning the years of 1821 to 1909 in English, German, and
French.
Device descriptions: A wide variety of devices were utilized to improve hearing and, purportedly, to reduce aural
discharge. The most popular devices were made of gutta percha attached to a silver wire stem (Toynbee) and cotton
balls with extraction cords (Yearsley). Other membranes included India rubber, lint, tin or silver foil, and even the
vitelline membrane of an egg. Adhesion to the drum remnant was with saliva water, Vaseline, or glycerine. Some
were applied by the physician, while others were inserted daily by the patient much like contact lenses are today.
Conclusions: In several cases, the method of positioning an object over the drum remnant was actually invented by
clever patients and then later adopted by practitioners. Once introduced, great optimism flowed about the
“miraculous” value of this deafness cure. Petty jealousy among early inventors led to very public (and
unprofessional) quibbles over the primacy of invention and bickering about whose device was superior. Over the
subsequent decades, as more experience demonstrated the device’s limited value, enthusiasm waned until otologists
largely abandoned these devices around the turn of the century. In the first two decades of the 20 th century, artificial
eardrums reached their peak of fame among the public when they were enthusiastically (and dishonestly) marketed
by a slew of quacks through newspaper ads as a universal cure of all forms of deafness. Only with the coming of the
FDA did ads for $10 mail order, medicated eardrums disappear from popular newspapers and magazines.
Robert K. Jackler, M.D.
Department of Otolaryngology
University of California, San Francisco
400 Parnassus Ave, A-730
San Francisco, CA 94143-0342
Bioactive Glass Ceramic Particles as an Alternative for
Mastoid Obliteration – Results in an Animal Model
Bryan D. Leatherman, MD, John L. Dornhoffer, MD
Hypothesis: This study was conducted to evaluate the use of NovaBone – C/M Bioglass® (Porex Corporation,
College Park, GA) as a graft material for mastoid cavity obliteration in an animal model.
Background: Canal wall down procedures in otologic surgery may result in a problematic mastoid cavity.
Mastoid cavity obliteration can potentially prevent or correct this problem. Many techniques and implant
materials have been utilized for mastoid obliteration, yet no single graft material has proven to be ideal.
Methods: Mongolian gerbils received tympanic bulla obliteration utilizing the NovaBone Bioglass particulate. Nine
weeks post-implant, the animals were sacrificed, and histologic sections were prepared. Histologic evaluation was
performed to evaluate new bone formation within the implant.
Results: Wound healing occurred without complication. Mature trabecular bone was found throughout the entire
thickness of the implant material. There was no histologic evidence of inflammatory reaction or short-term
resorption.
Conclusions: The high level of bone formation obtained using NovaBone in this study makes this material a
potential alternative resource as a graft material for mastoid obliteration in the future.
Bryan D. Leatherman, M.D.
Department of Otolaryngology-HNS
University of Arkansas for Medical Sciences
4301 West Markham, Slot 543
Little Rock, AR 72205-7199
Mastoid Obliteration: Autogenous Cranial Bone (Paté) Reconstruction
Joseph B. Roberson, MD, Katrina R. Stidham, MD
Theodore P. Mason, MD
Objective: To review outcome in patients with problematic mastoid cavities who have undergone primary complete
epitympanic and mastoid bowl obliteration over a 60-month period
Study Design: Retrospective review
Setting: Tertiary referral center
Patients: 68 sequential patients undergoing mastoid obliteration over a 60 month time period with major indications
including recalcitrant drainage and debris trapping in the canal wall down cavity, intolerance of water exposure,
vertigo that is calorically induced in the existing cavity, and inability to wear a hearing device. 34 patients
undergoing second stage surgery for ossicular reconstruction during the same time period are also reviewed.
Intervention: Transplanted autogenous cranial bone is used to induce osteoneogenesis resulting in complete
obliteration of the epitympanic and mastoid spaces while maintaining a mesotympanic space.
Main Outcome Measures: Success of obliteration, incidence of symptoms prompting intervention, hearing
outcome, incidence of recurrent cholesteatoma, incidence of eustachian tube dysfunction necessitating treatment and
need for revision surgical procedures.
Results: Complete take of the bony obliteration occurs in over 95% of cases. Over 90% of treated patients enjoy
complete absence of original symptoms. To date, no patient has required revision surgical intervention.
Conclusions: Mastoid obliteration with autogenous cranial bone is a safe and effective option for patients with
problematic canal wall down mastoid cavities. Surgical techniques that include sterile harvest of the cranial bone
graft mixed with antibiotic, revision of the cavity to expose viable native bone, and complete coverage of the (Paté)
with autogenous fascia have proven critical to successful outcome.
Joseph B. Roberson, M.D.
801 Welch Road
Palo Alto, CA 94304
Ionomeric Cement Reconstruction of Incus Erosion
Moises A. Arriaga, MD, Douglas A. Chen, MD
Objective: This study describes the technique and short-term hearing results of fast-setting ionomeric cement
(Serano-Cem) for managing incus erosion in revision stapedectomy and ossiculoplasty.
Study Design: Observational and retrospective chart review.
Setting: Patients were operated on an ambulatory basis in a tertiary referral center.
Patients: Consecutive patients undergoing ionomeric cement incus reconstruction during revision stapedectomy
and ossiculoplasty surgery.
Intervention: Therapeutic - Incus reconstruction with ionomeric cement.
Main Outcome Measures:
1. Technical details and recommendations for handling this new material.
2. Six-week hearing outcomes comparing preoperative and postoperative air
conduction and
bone conduction thresholds.
Results:
1. A small amount of ionomeric cement on the tip of otologic picks applied to the incus remnant successfully
reconstitutes the original length of the incus. In revision stapedectomy, a crimp-on prosthesis may be placed on the
cement-lengthened incus. In mobile stapes situations, the cement reconstruction directly bridges the ossicular
discontinuity produced by incus erosion.
2. Six-week postoperative audiograms demonstrate significant closure of the air-bone gap in operated cases.
Conclusions:
1. Ionomeric cement permits direct reconstruction of a pathologically shortened incus
in revision stapedectomy and ossiculoplasty.
2. Surgeons must be aware of precautions and limitations of this material.
3. Preliminary results indicate significant hearing improvement with this technique.
Moises A. Arriaga, M.D., FACS
420 East North Avenue, Ste. 402
Pittsburgh, PA 15212
Preliminary Experience with a New, Lightweight, Titanium
Prosthesis for Ossicular Chain Reconstruction
J. Douglas Green, Jr, MD, Clough Shelton, MD
John McElveen, MD
Objective: European studies have cited improvement in high frequency hearing results using a new, lightweight
titanium prosthesis. The effectiveness of this prosthesis in achieving this goal along with other traditional measures
of efficacy was assessed. Ease of handling, visualization and tendency for extrusion was also reviewed.
Study Design: Retrospective review of clinical cases.
Setting: Multicenter study (3 sites) involving academic and private practice tertiary care centers for otologic
surgery.
Patients: 166 consecutive patients undergoing otologic surgery requiring reconstruction of the middle ear sound
conduction mechanism across the 3 practice sites.
Intervention: Ossicular chain reconstruction both with and without mastoidectomy and tympanic membrane
reconstruction using a titanium total ossicular chain replacement prosthesis (T-TORP) and a titanium partial
ossicular chain replacement prosthesis (P-PORP). Cartilage was interposed between the tympanic membrane and the
prosthesis.
Main Outcome Measures: Audiometric data preoperatively and at 3 months, 12 months and last available
audiogram including pure-tone average, speech discrimination scores, and air-bone gap measures. Extrusion rate,
drop in sensorineural reserve and high frequency hearing were also studied.
Results: Postoperative air-bone gap of < 20 dB was obtained in 64% of patients having undergone placement of the
T-PORP. A postoperative air-bone gap of < 30 dB was obtained in 67% of patients with the T-TORP. Results were
stable over time in patients with long-term follow-up available. All three surgeons felt that the titanium prostheses
were easier to work with than other conventional prostheses, allowing visualization through the head of the
prosthesis, and all three now use these prostheses routinely. There have been no extrusions to date with a mean
follow-up of 9 months and a maximum of 27 months. High frequency hearing results were somewhat better than the
results presented above and were comparable to results from one of the European studies.
Conclusions: Initial experience with a new, lightweight titanium prosthesis demonstrated hearing results
comparable to other previous reports with improved ease of handling and visualization. Extrusion rates to date have
been acceptable. Improved high frequency hearing results over other traditional middle ear prosthesis is supported
by the present study.
J. Douglas Green, Jr., M.D.
Jacksonville Hearing & Balance Institute
836 Prudential Dr., Ste. 1405
Jacksonville, FL32207
Discussion Period 5, Papers 16 - 20
DR. CHOLE:
Thank you very much. These papers are now open for discussion, the panel discussion through
the other four papers of this session.
DR. JOHN MCELVEEN, RALEIGH DURHAM, NC,
First a comment. I’m really pleased to see people starting to use the bone cement. We have
been using the Otosom actually for what I call a conservation cholesteatoma surgery where we
are taking out the posterior canal wall, taking out the cholesteatoma and preserving the ossicular
chain and then using the cement to rebuild the posterior canal wall. We’ve tried other techniques
but now with the cement I think it makes it very useful. We always focus on conservation
hearing with acoustic neuromas. I think we need to start looking at conservation of the ossicular
chain with cholesteatoma surgery. The question I have for Dr. Ariaga is related to the incus to
stapes junction with this Ionameric cement. Do you make a solid connection to the capitulum or
do you try to recreate a little bit of the synovial joint and may this explain some of the problems
were having with that particular reconstruction:
DR. MOSIES ARRIAGA, PITTSBURG, PA
I guess two issues John, first with regard to the canal wall reconstruction. The Europeans
actually were using that as well as you know, Geiger, Helms and I think the one caution, more of
the facial nerve is exposed and being particularly cautious about that. With regard to the incus
reconstruction we are making a solid reconstruction and especially as we are learning more about
what happens to this material particularly early on with moisture. I don’t think the problems
with the incus reconstruction issues are related to a solid versus a synovial joint. I think the issue
is of eustachian tube problems and drum retraction in those cases. I think theoretically that’s
interesting. I know that in Tubegin they are doing it that way but I think we just need to
emphasize right now that with this material, dry is really important.
DR. JENNIFER MAW, SAN JOSE, CA,
Comment and caution for Dr. Leatherman and Dornhoffer. I commented at last year’s COSM
about some failures of mastoid obliteration with some of the new bone reconstruction materials
from Livinger. I just want to elaborate that I have seen a lot more failures and I will be
presenting that paper next year but I’m up to a 70% failure rate using those types of
reconstruction materials in the mastoid covering it with the traditional fascia graft as described
by Dr. Roberson . So just go very slow with your clinical trial because I think the glass products
are even less likely to be accepted in the mastoid and my failures have been very late, eighteen
months to two years so go very cautiously with your clinical trials with this. Thank you.
DR. JAN GROTE, THE NETHERLANDS:
I have the same problem and I have remarked to the presentations of Leatherman and Roberson.
Most dealing with new bone formation. Now the problem of course is how to get a reliable
scaffold in order to promote new bone formation. At the end of the 70’s I published a lot of
papers on that. Both materials presented are bioactive so that’s alright. The problem however is
different for both materials as already mentioned. For the bone pate it looks like good material
but the porosity is too high. We know exactly what kind of porosity you need, in order to have a
reliable new bone formation before it’s resolved because you don’t want to end up with holes.
That is why I introduced hydroxlappetite and developed it. In 83 I finally agreed that it came
into the clinic. With the bioglass, the problem we already know is that the outer surface is the
calcium phosphate iron, which will get new bone formation . This center is still a foreign body
bioglass so in the long run it will resolve and that’s why Cerventol was taken from the market.
Now my question to Leatherman, he didn’t see any inflammation but did he look at the
microphage reactions in his experiments because that will indicate that there will be a resorption.
As already mentioned by the question before that there will be resorption in the long run.
Microphage reaction will continue to beat out the bioglass, that’s why I ask this question as to
whether you have seen that or not?
DR. BRYAN LEATHERMAN, LITTLE ROCK, AR
Yes sir, no we didn’t particularly look at the macrophage reaction. The reason being is the
particles all reabsorb part of the process. The bone forms on the outside and works towards the
middle. There’s been histological studies of this material carried all the way out to no crystals
left. There’s no crystals exposed for microphages to come so even if there’s crystals in the
middle ear there’s no exposure for macrophages to come in contact with them. In previous
studies we didn’t look at that because it’s been looked at and eventually all the material does get
reabsorbed and its supposed to as the bone gets formed around it.
DR. EDWARD BRANDON, SYRACUSE, NY
Yes, I enjoyed the papers on mastoid obliteration but a word of caution. There’s an incidence of
recurrent cholesteatoma beneath the obliteration flap. It’s alright to obliterate a relatively stable
old mastoid cavity but in the face of active cholesteatoma disease you probably better not
attempt this. A few years back Beels from England poled over a thousand otologists concerning
the results of pedicle flap obliteration and he found an incidence of 5% cholesteatoma
recurrence under the flap. He also found three cases of brain abscess secondary to the
obliteration.
DR. ILMARI PYYKKO, STOCKHOLM, SWEEDEN,
I have a question for Dr. Roberson about the use of bone pate in the mastoid obliteration. We
have been using that, as many of you, in Europe on just using the bone pate. My first question in
closing the fistula is causing long-term bacterial phenomena for the patient because it’s not
forming the solid bone in these cases and we have to change the technique and use bone chips for
that. The same holds for long-term results. If we look for ten years follow up for these patients
for the canal reconstruction. It seems that in significant parts of the cases there is continuous
absorption of the canal so therefore we use additionally bone chips for the canal which are more
stable. I would like your comments on these two topics.
DR. JOSEPH ROBERSON, PALO ALTO, CA
Thank you for your comments and questions. In terms of the fistulas what we have done is
placed fascia over the top of the fistula and then placed a large amount of bone to fill the mastoid
cavity. To date those patients have experienced a mild disequilibrium for a few weeks after
surgery but following that they have been in good shape. They do not have any sort of vertigo
induced either with pressure in the canal and it seems to have worked nicely. Your comment
regarding I think really a synopsis is the size of the bone that has moved into place either small
pieces of bone taken with the drill like bone pate or bone chips. We have done some work with
ear canal, actually my partner Dr. Rodney Perkins had done some work early with reconstruction
of the ear canal and his findings were similar. Our switch in the last several years has caused our
bone pate to be more coarse and my clinical impression although I can’t show this is that does
seem to be a more stable compound if that’s right. We have switched from a low speed to a high
speed drill. The flutes of the drill bits are more aggressive and the chips are larger so there may
be something to that.
DR. ANTONIO DE LACRUZ, LOS ANGELES, CA
I have a question about the panel. In the first case that you presented and I don’t know if John
Niparko was here, the comment on the bilateral acoustic NF2 in a child. Was this the two cases
facial function in those cases. That was the impression I got.
He resected the tumor from the nerve and preserved facial function.
DR. ANTONIO DE LACRUZ:
We have looked at the histopathology of NF2, that is very hard to do.
DR. CHOLE:
I agree.
DR. JOHN HOUSE:, LOS ANGELES, CA,
I would like to first of all make a comment on Eugene Chu’s talk and Bob Jackler. That was
very enjoyable, you did a great job presenting it and I enjoyed it very much. Of course I always
like history; I think its always interesting to look back on what’s been done in the past. A
comment and question for Joe Roberson. Joe, as you know we have been doing the bone pate for
many, many years and I think Rod started it even before that so it’s been a long time. I was
curious what are you using to collect the pate, using the Sheehy pate collector and the second
question is that I find it helpful to swing a little Palva flap down to get a little more soft tissue
over the bone pate. I am wondering if you do that?
that he had that were facial nerve neuromas and did he resect them and save the hearing? It kind
of concerned me a little bit because most of us would have to open the internal canal realizing
that it was a facial nerve neuroma not resect, leave it on and let the patient have normal facial
function for as long as they could. So I was just wondering if he resected and grafted the facial
nerve right off the bat and that was my question to him.
DR. ROBERSON:
Yes, we do use the Sheehy bone pate collector although in hospitals where we don’t have that we
have collected the pate with the tongue blade and with a burr rotating into that the old fashioned
way. Your second question was regarding the Palva flap. I have not done that. I think the
reason I like the way we do it better now is that the fascia is a thickness that is uniform and
there’s not the variability of how much soft tissue reabsorption there is. It’s also less traumatic
to the ear if not to move so much tissue around. We do retain and these slides didn’t do it
justice, flaps of skin anteriorly and posteriorly. We reflect those onto the anterior canal wall and
leave them there, obliterate the mastoid and then cover the fascia with that but have not used
tissue over the top of it.
DR. HERBERT SILVERSTEIN, SARASOTA, FL,
I would like to comment on the paper the Ionomeric cement reconstruction. I just wanted to
caution the audience. We just had experience using this cement on a very simple case with incus
necrosis connecting the incus to the stapes and we waited for the cement to dry and then did a
tympanoplasty to reconstruct the eardrum putting gel foam and Vasociden in the middle ear. The
patient developed a severe sensorineural hearing loss right after the surgery. We placed the
patient on a high dose of steroids and the sensorineural loss improved to the point where the
patient has about a 50 -decibel loss and about a70% discrimination. I reported this to the
company. They are doing research on this cement and it is quite toxic and they are
recommending even waiting 20 minutes before doing any other kind of work with the cement.
We imagine that what happened was that there must have been some reaction with the cement
and the Vasociden going down to the round window. When we took the pictures and the video
we could see it was all connected together and it looked like the toxic substance went into the
inner ear to cause the sensorineural loss so I caution people using this cement.
DR. JIM SAUNDERS, OKLAHOMA CITY, OK
Joe, I wanted to make a comment really not so much a question but in your response to Dr.
House. I share your concerns about the palva flap have had some valuable reabsorption of that.
What I have been doing with my canal wall reconstruction is actually just taking the periostium
on the undersurface of the palva flap which swings into the ear very nicely and gives you a nice
vascular coverage without that really big thick flap which you sort of end up with a lot of thick
tissue laterally at the meatus and not much deep so it might be something you can consider
trying.
DR. CONRAD PROCTOR, DETROIT, MI,
I followed about 5000 cases of my late father and I did over 25 years using the open cavity
technique and our results are very good. Only about 1% of these have needed revision and only
about 1% have recurrent drainage so I’d like to just put in a word for the old technique. Probably
the secret was the way the mastoidectomy was done. My father taught anatomy and I learned a
lot from him and at the University of Michigan and if the mastoidectomy is done properly,
results are fantastic I would say.
DR. CHOLE:
Thank you Dr. Proctor. One last word from Dr. Grote..
DR. IAN GROTE, THE NETHERLANDS,
A remark on the periosteal flap. I used the cranial base periosteal flap and not the Palva flap.
The reason for that is that with the Palva flap you put it outside of the flap on top of the new
bone formation and the inside of the flap is the bone inducing part. So you really enhance the
new bone formation if you take the underside of the periosteal flap on top of new bone
formation. That’s why I use the cranial base.
DR. CHOLE:
Thank you. Thank you all for your excellent discussion.
Middle Ear/Mastoid
Diagnostic Utility of Laser Doppler Vibrometry in Conductive
Hearing Loss with Intact Tympanic Membranes
John J. Rosowski, PhD, Barbara S. Herrmann, PhD
Ritvik P. Mehta, MD, Saumil N. Merchant, MD
Hypothesis: Laser Doppler Vibrometry can help the diagnosis of conductive hearing loss.
Background: The identification of pathologies responsible for conductive hearing loss with intact tympanic
membranes is a continuing challenge. While history, audiometry and tympanometry are helpful, the precise
diagnosis is often made only during surgical exploration. The dependence on surgery for proper diagnosis increases
the difficulty of pre-surgical counseling and planning. The present study investigates the utility of laser vibrometry
in conductive diagnoses.
Methods: Pre-surgical laser vibrometry was used to determine the sound-induced vibration of the umbo in 16 cases
with conductive hearing loss, intact tympanic membranes and aseptic middle ears. Sound was delivered to the ears
via a glassbacked speculum that allowed us to visualize and focus the laser on the umbo. The difference between
the measured vibration and the vibration in normals was compared to the surgical diagnosis. The surgeon was
blinded to the laser results. Post surgical umbo vibration measurements were also made when a stapedotomy was
performed.
Results: Three cases with ossicular interruptions had significantly larger than normal pre-surgical umbo vibrations
at low frequencies. Thirteen cases with ossicular fixations had smaller than normal umbo vibrations. The umbo
vibrations measured post stapedotomy where similar to the measurements made with ossicular interruptions.
Conclusions: The results show differences in sound-induced umbo vibration between cases of ossicular interruption
and fixation that may be useful in the differential diagnosis of these conditions. Post-stapedotomy measurements
suggest successful stapes surgery produces a more mobile middle ear than normal.
[Supported by NIDCD; IRB Approval 00-09-041].
John J. Rosowski, Ph.D.
Eaton-Peabody Laboratory
Massachusetts Eye & Ear Infirmary
243 Charles St.
Boston. MA 02114
Middle-Ear Mechanics of Type III Tympanoplasty: Basic and Clinical Studies
Ritvik P. Mehta, MD, Michael E. Ravicz, MS
John J. Rosowski, PhD, Saumil N. Merchant, MD
Objective: To investigate mechanics of type III tympanoplasty.
Background: In the classical type III-stapes columella tympanoplasty (graft placed directly onto stapes), postoperative air-bone gaps vary from 10-60 dB. The structural features responsible for the wide range in results are not
well understood.
Methods and Results:
(1) Experimental study: Canal wall-down type III procedures were performed in 10 cadaveric temporal bones.
Acoustic stimuli were presented in the ear canal, and round window velocity VRW (used as an index of hearing) was
measured, while systematically varying stapes mobility, graft material and graft-to-stapes coupling.
VRW after a type III was 20-25 dB lower than in normal ears. Stapes fixation markedly reduced VRW. There was
little effect of varying the graft material or varying the tightness of coupling between the graft and stapes.
Interposing a thin cartilage disc between the graft and stapes improved VRW by 5-15 dB.
(2) Clinical study: Post-operative air-bone gaps were determined in 40 ears after type III tympanoplasty with
temporalis fascia graft onto the stapes head. Air-bone gaps of 20-25 dB occurred with a mobile stapes and aerated
ear (N=26), which corresponds well with the temporal bone results. Large air-bone gaps of 40-60 dB occurred with
stapes fixation (N=4) or non-aerated ears (N=10).
Conclusions: A mobile stapes and aerated middle ear are essential for a successful type III tympanoplasty. There is
little effect of varying graft materials or graft-to-stapes coupling. Improved results may be achieved by interposing
a thin cartilage disc between the graft and stapes head; we hypothesize that the disc improves the "effective"
vibrating graft area.
Supported by NIDCD R01 DC04798; IRB approval #00-09-041.
Saumil N. Merchant, M.D.
Department of Otolaryngology
Massachusetts Eye & Ear Infirmary
243 Charles Street
Boston, MA 02114
Prolonged Middle Ear Ventilation with the Cartilage Shield T-Tube Tympanoplasty
Larry G. Duckert, M.D., Ph.D., Kathleen H. Makielski, M.D., Jan Helms, M.D
Abstract: In 1994, the favorable experience with composite cartilage “shield” tympanoplasty was reported to the
Society. On that occasion, the technical question regarding the concomitant placement of a ventilating tube was
posed. In response, the authors proposed that the tympanostomy tube be incorporated in the cartilage graft.
Moreover, they proposed that this marriage, when used to reverse atelectasis repneumatize the middle ear, should
offer the advantage of both procedures while reducing the incidence of tube extrusion and other complications of
prolonged intubation. While attractive in theory, this supposition could only be validated after a prolonged period of
follow-up, reported here.
Objective:
1. To describe an effective means to secure prolonged middle ear ventilation in a patient
population prone to atelectasis and chronic middle ear effusion; and
2. To establish the incidence of favorable and unfavorable outcomes after six years of
observation.
Study design: A retrospective case series.
Setting: A tertiary referral center.
Patients: 40 patients (28 adults and 12 children) who underwent tympanic membrane reconstruction with
a composite cartilage shield T-tube unit.
Results:
1. Overall retention rate was 62.5% over 6 years;
2. 65% of retained tubes were maintained a minimum of 4 years in
adult patients; and
3. Extrusion and permanent perforation rates were 0%.
Conclusion: The cartilage shield T-tube tympanoplasty can effectively reverse atelectasis and provide prolonged
middle-ear ventilation. The technique can be used safely and minimizes the risk of tympanic membrane perforation
and other complications associated with prolonged middle ear intubation.
Larry G. Duckert, M.D., Ph.D.
Otolaryngology-HNS
University of Washington
Box 357923
Seattle, WA 98195-7923
Laser Eustachian Tuboplasty (LET)
Oskar B. Kujawski, MD, Dennis S. Poe, MD
Objective: Surgery on the eustachian tube (ET) for chronic eustachian tube dysfunction (ETD) has been previously
directed toward the bony isthmus and failed to produce lasting results. Dynamic video analysis demonstrates
pathophysiology in the tubal cartilaginous portion. This study investigated a new endoluminal procedure that
focused on the cartilaginous ET.
Study Design: Prospective surgical trial.
Setting: Tertiary care private practice and outpatient surgi-center.
Patients: 108 ETs with intractable ETD (middle ear atelectasis or effusion) in 56 patients underwent LET by first
author OBK since 1997.
Intervention: Dynamic video analyses of ET function were done peri-operatively. LETs were performed
unilaterally or bilaterally under general anesthesia through a combined endoscopic nasal and trans-oral approach to
the ET nasopharyngeal orifice. CO2 or 980nm diode laser vaporization of mucosa and cartilage from the lumenal
posterior wall was accomplished until adequately dilated and the tube was packed.
Main outcome measures: Opening of tubal aperature on dynamic video. Normalization of mucosal edema,
impedance tympanometry, and tympanic membrane.
Results: 74 (69%) tubes achieved normal ET function at 1 year, 70(71%) at 2 years, and 60(65%) at 3years. There
were no intraoperative complications. Post-operative complications were limited to minimal peritubal synechiae in
9 (8.3%)tubes and epistaxis in 1 (0.9%) tube. 7(6.5%) tubes failed treatment and required tympanostomy tubes.
Conclusions: LET is a new procedure that has demonstrated early promise in correcting intractable ETD with little
complications. Further studies will be necessary to reproduce the results and establish the role of LET in the
management of chronic intractable ETD.
Oskar B. Kujawski, M.D.
3, rue du Conseil-General
1205 Geneva, Switzerland
Photoacoustic Effects of Carbon Dioxide Lasers in Stapes
Surgery: Quantification in a Temporal Bone Model
Edward K. Gardner, MD, John L. Dornhoffer, MD
Scott Ferguson, ALET
Hypothesis: The carbon dioxide (CO2) laser in stapes surgery creates sound
waves that could damage hearing.
Background: Application of a laser to any medium causes absorption, reflection, transmission, and thermal effects.
To date, research on the safety of CO2 laser stapedotomy has focused on the thermal effects. However, its
absorption also presents some risk to the inner ear as the absorbed energy elicits photoacoustic or photochemical
effects. Our goal was to measure the photoacoustic effects (sounds) produced by the CO2 laser in simulated stapes
surgery.
Methods: Using a variety of settings, a Sharplan 150 XJ Laser and a Contour Erbium:YAG laser were applied to the
oval window of human temporal bones fixed in a normal saline bath to simulate perilymph. Photoacoustic waves
were measured by a hydrophone located 2 mm beneath the oval window. Measurements were made with and
without a simulated tissue seal over the window.
Results: No detectable sounds were created below 4 Watts (continuous mode) or 60 mJ (superpulse mode). Above
these settings, intensities greater than 90 dB were detected when the laser was applied directly to the perilymph.
With the tissue seal in place, no detectable sounds were identified. The accuracy of our model was confirmed by
comparing our results with previously published results using the Erbium:YAG laser.
Conclusions: Below 4 Watts in continuous-wave mode and below 60 mJ in superpulse mode, any sound generated
by the laser is negligible. Above these thresholds, however, impact sounds are produced that could result in
threshold shifts with repeated applications.
Edward K. Gardner, M.D.
4001 West Capitol
Little Rock, Arkansas 72205
Physiologically Optimal Placement of Malleus-to-Footplate Prosthesis
Larisa D. Kunda, MD, Sunil Puria, PhD
Joseph B Roberson, Jr., MD, Rodney C. Perkins, MD
Hypothesis: To physiologically optimize malleus-to-footplate prosthesis (MFP) reconstructed ears.
Background: The positioning of MFP on the malleus remains a controversial subject. In the past, the acoustic
properties of the MFP have been studied using a laser Doppler vibrometer system. Recently, it has been shown that
hearing threshold is better correlated to cochlear pressure. We measure cochlear pressure to study human cadaveric
ears reconstructed with hydroxylapatite MFP.
Methods: The ear-canal pressure (Pe) and vestibule pressure (Pv) are measured in four temporal bones with
additional measurements planned. In each bone, measurements were performed (1) with intact incus, (2) removed
incus, and (3) with MFP at three positions on the malleus (head, mid-manubrium, and umbo) and center of footplate
with intact and removed stapes-superstructure (SS). The incus gain (Gi), is defined as the ratio of Pv before and after
removing the incus. Similarly, the MFP gain (Gmfp) is defined as the ratio of Pv with reconstructed MFP and
removed incus.
Results: Gmfp reached 37-38 dB with MFP at the neck and with the SS intact and after removal. The range of
Gmfp at the mid-manibrium and umbo, with SS intact and after removal, was 23-26 dB. For reference Gi was 42 dB
for all ears. All of these averaged peak gains were in the 1.1 to 1.3 kHz region.
Conclusions: With MFP reconstructed middle ears there is a 11-15 dB improvement in pressure gain with the
prosthesis beneath the neck of the malleus than at other locations.
Larisa D. Kunda, M.D.
738 Channing Avenue
Palo Alto, CA 94301
Discussion Period, Papers 21 -26
DR. GULYA:
Okay, these papers are now open for discussion. Dr. Mansfield Smith.
DR. MANSFIELD SMITH, DAVIS, CA,
I would like to comment on the excellent paper on Photoacoustic Effects of Carbon Dioxide
Lasers in Stapes Surgery. We developed a number of years ago a CO2 laser delivery system
with Reliant Laser, which used a mirror reflection system, which did two things. One, it allowed
the laser, using CO2 because of all the reasons given,and it brought the laser aiming beam and
the laser so they are exactly the same. Two, that the laser beam could be reduced to a tenth of a
millimeter accurately which makes it a much more effective instrument. It reduces the energy
delivered particularly noise and with the absorption of it less than a tenth of a millimeter into
perilymph it made it extremely safe. I’ve used that for years and I’m glad to see this work
further collaborate that effort. Thank you
. DR. GEORGE LESINSKY, CINCINNATI, OH,
I would again like to congratulate John on a wonderful paper but I need to point out several
important facts. One is the laser company’s deceived us when we got to pulsing CO2 lasers by
settings called “average power”. Milligules are terribly important when we are pulsing the laser
and the milligule is defined as the watts times the milliseconds that the pip of the micropulse is
on. A micropulse might be on for a tenth of a second but it might have ten pips that are only a
tenth of a millisecond. Our work shows that anything over 25 milligules per pip is dangerous
because we get flaring. We had recommended in our work with the thermal effects between 10
and 20 milligules per pip. IL Med Laser that was developed specifically for CO2 laser has 16
milligules per pip. The problem is the neurosurgeons and other specialists who are driving this
CO2 laser force Sharplin and Coherent to be delivering 500-600 watts of peak power while they
were on super pulse so the least you could get was 50 milligules which was much too powerful.
It is terribly important when we are revising stapes surgery to open the vestibule. It is the only
way I know of to determine the true depth of the footplate where it should have been and also
any residual footplate below it and many, many times as I pointed out in a paper last year where
we reviewed 289 I believe revisions, the collagen layer contracts and is above the level of the
footplate. So palpation of the sealing collagen neomembrane is not valid because we are not
near the footplate. It lateralizes up on the promontory and facial ridge and consequently in 18%
of the cases we have found intact stapes footplate below the center of the fenestration. In
summary milligules per pip is what’s important. The thermal spread is dependent on how long
the power is on the target tissue and if we use continuous wave for a tenth of a second or were
using super pulse, the difference is the super pulse pip is on for a tenth of a millisecond, 1000
times less than a continuous pulse. In revision surgery if you do revision under local like I do
that is important if we raise the temperature of the vestibular perilymph and ultimately
endolymph more than one degree centigrade we can begin to get vertigo. In summary super
pulse mode is the best mode for stapedotomy and stapedectomy revision, calculate the milligules
per pip on super pulse mode by multiplying the on time for the super pulse mode each pip times
the peak power and keep between 10 and 20 milligules.
DR. ELIAS MICHAELIDES, DETROIT, MI,
A question for Doctor Dornhoffer. You noted that at lower power settings you got no decibels
and as you raised up the power levels you did get a response. Could you explain how you were
getting absolutely no sound levels at the lower power settings and were you seeing any tissue
effect at those power levels?
DR. DORNHOFFER:
It has to do with energy. I mean the photoacoustic effect is probably created by a small bubble
of vaporized medium and it’s just less energy. I think that’s just the best way to express it. It
may be simplistic but I think it didn’t produce detectable sounds also which means that it doesn’t
necessarily mean it produced no sound, it just means that we weren’t able to detect them with our
current setup if that answers your question..
DR. JENNIFER MAW, SAN JOSE, CA:
I enjoyed the papers on the eustachian tube problems very much despite all the neurotology
training, I’m still humbled it seems just by simple eustachian tube dysfunction. I’m wondering if
Dr. Duckert could give us some words of wisdom about keeping the tubes open after surgery
because that’s been a problem for me. and if Dr. Kujawsky could comment in a chronic sinusitis
patient is pus in the nasal pharynx a contraindication to doing a procedure?
DR. OSKAR KUJAWSKY, GENEVA, SWITZERLAND,
First your question is very important. I mentioned before we consider that with any surgery all
factors that can lead to obstruction of eustachian tube has to be removed or try to be treated.
What you mentioned evidently is one so the first cases I’ve done with a careful selection. They
were divers and they just wanted to be better, I said okay we will try so of course we have to
resolve all pathologies known before. I won’t say it’s a contradiction but if you don’t treat it
before or consider the correct way to approach this very new early surgery.
DR. LARRY DUCKERT, SEATLE, WA,
Thank you for the question regarding patency of the tube. That is a problem technically
speaking. At the time of surgery the less amount of gel foam that you put in the middle ear which
has a less tendency to obstruct the tube more acutely. Later on passage of very small suction tips
can facilitate or increase or maintain the patency of the tube. That’s a problem we have in
children. I think that probably contributes to the increased failure rate in children because they
don’t tolerate the tubule maintenance and manipulation of the tube. They have a greater
incidence in those situations where the tube has to be removed but keeping it clean with a suction
tip does seem to help
DR. ILMARI PYYKKO, STOCKHOLM, SWEEDEN,
I have one question for Dr. Kujawaski.. I think this new approach is really promising but when
we are doing endoscopy of the eustachian tube we see a different kind of pathology like mucosal
pathology which you can easily access with your laser surgery. In some rare cases there may be
ankylosis of the cartilage prohibiting it from rotation. The third pathology which seems to be
present is the muscular pathology. My question is do you think that those cases which were not
responding for later surgery were belonging to muscular pathology of the eustachian tube or
what is your opinion on that?
DR. OSKAR KUJAWSKI,
I didn’t want to mention in this study old data base that I’ve taken to consider patients. Slow
motion video helped me before to overall see if there is a functional, a more obstructive
permanent problem. There are some first results but I prefer just Dr. Poe who has more focus
now on this dysfunction or obstruction of eustachian tube, which is a crucial problem of course.
We have some enhancement with the function of the obstruction that maybe we can say is an
obstruction problem to delay the eustachian tube, maybe this is a correct word so those data are
really evident, they are the first. The criteria is very easy, very simple. There is quite a good
data base but we need much, much more data and before we analyze the results we have really to
discard old data. I had to learn a lot before I imagined the surgery. So the discussion is very
open.
DR. ARVAND KUMAR, CHICAGO, IL,
The question is for Dr. Kunda. Did you measure the angle between the handle of the malleus
and the center of the footplate because that would influence how the system works:
DR. LARISA KUNDA, PALO ALTO, CA,
Unfortunately we did not come up with any good measurement devices for that but we do plan to
do it in the future.
Vestibular
Efficacy of a Falls Prevention Clinic: A Pilot Study Utilizing Quality of Life Assessments
Priya Krishna, MD, Manali Amin, MD, Marian Girardi, MA
Sandra Lin, MD, Horst R. Konrad, MD, Larry F. Hughes, PhD
Objective: To determine whether evaluation at and intervention from a Falls Prevention Clinic improves balance,
reduces falls risk, and affects quality of life for elderly patients.
Study Design: Retrospective chart review
Setting: Tertiary referral center
Patients: Urban/rural Midwestern elderly population referred to a Falls Prevention Clinic.
Intervention: Rehabilitative
Outcome Measures: Dizziness Handicap Inventory and Investigator-Designed Survey.
Results: Thirty-six patients who were evaluated and treated at the Falls Prevention Clinic were asked to participate
in this pilot study. 100% of these individuals had fallen at least once prior to visiting the clinic, and their mean
number of falls was 3.36. All were given specific customized therapeutic balance exercises to perform. A large
majority of patients performed the exercises. Approximately two years after their initial evaluation and treatment at
the Clinic, these individuals completed Dizziness Handicap Inventory (DHI) forms and investigator-designed
surveys. The mean age of the patients was 80.4 years. In the two years since their visit to the clinic, only 23.1% of
the respondents reported falling and no serious fall-related injuries were described. DHI's completed post-treatment
demonstrated a statistically significant improvement to DHI's completed pre-treatment (p=0.026). In addition, the
satisfaction level with treatment was very high, with almost all patients stating they would recommend this Clinic to
other individuals with similar balance, dizziness, and falling problems.
Conclusion: Elderly patients who received therapy at a Falls Prevention Clinic were satisfied with their experience,
demonstrated both objective and subjective improvement in their symptoms of dizziness and imbalance, showed a
decrease in falls, and reported an improvement in their quality of life.
Marian Girardi, M.A.
Division of Otolarlyngology
SIU School of Medicine
P. O. Box 19662
Springfield, IL 62794-9662
Anatomic Differences in the Vestibular Canals and their Implications in Vestibular Neuritis
Gerard J. Gianoli, MD, Joel A. Goebel, MD, Sarah Mowry, BS, Paul Pooms, BS
Hypothesis: Anatomic differences may render the superior division of the vestibular nerve more susceptible to
injury during vestibular neuritis.
Background: Neural degeneration has been identified in temporal bone studies of vestibular neuritis. Previous
studies of vestibular neuritis have demonstrated that the superior division of the vestibular nerve is preferentially
affected, with sparing of the inferior division. A preliminary temporal bone study has implicated neural entrapment
as a possible cause for this preferential injury.
Methods: Two independent observers performed histologic analysis of 184 normal temporal bones from our
temporal bone library. Measurements of the medial, lateral and midpoints of the superior vestibular, inferior
vestibular and the singular nerves and canals were made. These measurements included the length, width and
percent of the canals occupied by bony spicules.
Results: The lengths of the bony channels of the singular nerve (0.598 mm) and the inferior vestibular nerve (0.277
mm) were significantly shorter than the average length of the superior vestibular canal (1.944 mm; p<0.0001). The
total percent of the canal occupied by bone at the canal midpoint was significantly greater for the superior vestibular
canal (28%) compared to either the singular canal (0%) or the inferior vestibular canal (18%) (p<0.0001).
Conclusion: The bony canal of the superior vestibular nerve is seven times longer than the inferior vestibular canal
and four times longer than the singular canal. There are a larger percentage of bony spicules occupying the superior
vestibular canal compared to the inferior vestibular or singular canals. This anatomic arrangement of a longer canal
with more interspersed bony spicules would make the superior vestibular nerve more susceptible to entrapment and
ischemia.
Gerard Gianoli, M.D.
17050 Medical Center Drive, Ste. #315
Baton Rouge, LA 70816
Meniett Therapy for Meniere’s Disease
George A. Gates, MD, J. Douglas Green, MD, Richard J. Wiet, MD
Educational Objective:
At the end of this presentation, the participants should be able to describe the short-term benefits of Meniett therapy
for people with medically unresponsive unilateral cochleovestibular Ménière’s disease.
Objectives: To evaluate the use of the Meniett device for treatment of people with unilateral cochleovestibular
Ménière’s disease who have failed medical therapy.
Study Design: Descriptive preliminary analysis
Methods: Intermittent transtympanic micropressure therapy using the Meniett device was self-administered for 5
minutes 3 times a day. The Meniett generates complex pressure pulses with a total amplitude of 12 cm. H 20 and a
duration of 0.6 sec. The micropulses reach the round window via an indwelling tympanostomy tube.
Results: Eleven consecutive patients with classic unilateral Ménière’s disease received Meniett therapy. Ten cases
were judged as responders. Cessation of vertigo and relief of pressure symptoms occurred within 2 weeks in 5 and
within 2 months for a sixth. The remaining patients estimated reductions in vertigo frequency and intensity of 50%.
One patient failed to respond in three weeks and underwent endolymphatic sac surgery. Improvement in hearing was
confined to the low tones and was modest. The treatment was well tolerated and no complications were observed.
Conclusions: This preliminary experience indicates that the Meniett is a reasonable option for people with classic
unilateral Ménière’s disease who have failed medical therapy.
J. Douglas Green, Jr., M.D.
Jacksonville Hearing & Balance Institute
836 Prudential Dr., Ste. 1405
Jacksonville, FL 32207
Discussion Period, Papers 27-29
DR. ALLEN RUBIN, TOLEDO OH,
I just wanted to address my question to George Gates on the Meniett device. Have you noticed
any difference in the outcomes based on the duration of the patients who have had Meniere’s?
That’s my first question, the second question any difference in the age, do the younger patients
do better than the older patients and the third question is do you think there will be a difference if
you use something like a T-tube with a larger boar than the smaller tubes?
DR. GEORGE GATES:
Thank you Allen. We don’t have enough people to analyze and answer the question about age
and duration. The preliminary feeling is that people with shorter duration Meniere’s disease
whose hearing fluctuates in between attacks will do the best. The type of tube I think is going to
be very important. We are using a larger bore gromet. Dr. Densert did not recommend T-tubes
but I think that’s going to be an option in the future.
DR. LOREN PARNES, LONDON, CANADA:
This is also for Dr. Gates. I know that in one of the European studies it was noted that patient’s
attacks lessened after the insertion of the tube and there was no subsequent improvement once
they started using the pressure device, the Meniett device. I was wondering if you noted that in
your group of patients?
DR. GEORGE GATES:
Thank you. We have not noticed improvement with the tube but we haven’t looked at it
specifically. We will in the clinical trial. Dr. Oldquists group had a interval between tube
insertion and use of the placebo and control device and there was no effect of tube insertion on
these patients symptoms. So we believe it’s the device and not the tube.
DR. JOHN HOUSE, LOS ANGELES, CA,
George one more question for you. A question about the tube, you said that you shouldn’t do it
without the tube being in place and how do you know if the tube does come out because in my
experience of one case the tube did in fact come out. He continued to use it. They came back
because the symptoms sort of returned but you implied that there’s a contraindication to use it
with an intact tympanic membrane
.
DR. GEORGE GATES:
Yes thank you. We have to teach the patients how to valsalva and clear their tube and we ask
them to check that they can feel air coming out before every use. Barbara Densert who is one of
the inventors of the device notes that when the tube is blocked and people use it, it can make
them worse. I don’t know why it makes them worse. The theory is that the pressure application
is going to the round window and not through the ossicular chain. There’s a lot to learn on this
yet.
DR. LARS OKAVIST, SWEDEN,
I was referred to and I have to make a few comments. Once you take a Meniere’s patient into
your service you really have to treat the patient all the way until for example you do the nerve
sectioning or the gentamycin and you have at last treated the patient for the final solution.
Concerning the function of the tube. We have a certain number of patients in this two week
study that was mentioned and also in the ongoing study with two months of treatment and half of
the patients for the placebo.. There are about 10% of these people who get rid of their attacks by
tube treatment alone. As we know from the publications by Tumarkin in the 60’s already so
there is an effect in some patients. Also in these studies we don’t accept the patients who get
well on the tube alone so those are excluded.
DR. FRED TELESHI, MIAMI, FL,
Why can’t patients generate the same effect with simple autoinflation?
DR. GEORGE GATES:
That’s a good question. When I was talking to Jack Hough about this he reminded me that when
he was a resident eustachian tube inflation with a cannula was a useful
therapy and widely used to abort impending Meniere’s attacks. This may be an alternative but
not all people can do that and it hasn’t been studied in a way that is scientific. One of the points
that have been made is that static pressure does not change the fluid distribution in the ear very
much and the Densert’s did a number of studies on this. It’s the alternating pressure so I suppose
if you can pop and push and pop and push you might get some therapy but that appears to be a
key element.
DR. GEORGE HICKS, INDIANAPOIS,IN,
Doctor Gates one of my associates late yesterday afternoon was at the Medtronix desk here and
was told that the company is going public for all physicians to have access to the device. I don’t
know if that’s true or not and if so the ramifications could be not desirable. What is your opinion
and is this a true fact?
DR. GATES:
Well the device has been cleared by the US FDA for sale and I understand that the Swedish
company Pascol Medical was purchased by Medtronix Xomed and that once the manufacturing
gets up to speed in the United States it will be available for any physician on a prescription. I
think that the use of this will be limited to a otolaryngologists and otologistss who know how to
put tubes in the ears, diagnose Meniere’s disease and monitor and care for these people. These
are high maintenance patients and it takes a lot of your time and effort to counsel with them and
walk through this to check that the machine is working because the patients get panicked when
the machine stops working and the emails and phone calls come back and forth. So there is a
substantial physician overhead on this.
DR. FIRAS HAMDAN, PERRY, FL,
Last question to follow up on the autoinflation, the flip side of it. Would you advise the use of
this device on a patient with patulous eustachian tube or would you skip it and go along for other
options?
DR. GATES:
I don’t think the patulous eustachian tube would affect it but I don’t know that.
DR. JOHN LI, JUPITER, FL,
What is the science behind the one second on and four seconds off or is there science behind that
and how much pressure and all of that kind of stuff?
DR. GATES:
Well if you read Dr. Densert’s articles over the past twenty years, there are a number of carefully
done animal experiments to examine various combination of pressure pulsed with intensity. I
would refer you to those articles for an answer to your question. This is the result of those
investigations.
DR. STEPHANIE MOODY, LOS ANGELES, CA
It’s interesting the effect on the dizziness but do you have any insight whether the device is
altering the pathophysiology of the disease such as the hearing loss that progresses over time?
DR. GATES:
In some cases it appears to alter the pathophysiology as the hearing improves and the vertigo
disappears. Some of our patients have stopped using the device three times a day and then go
down to two and then down to one and then their vertigo recurs and they get back on the device.
There are a number of mysteries involved in this and that’s why I refer to the black box and I
think that continued research will help answer a lot of these questions. The main point at this
time is there is an effect from the device; it appears to be a continuing effect as long as it’s used
and the device seems to be safe and reasonably effective.
DR. GULYA:
With that I would like to thank the speakers for staying to their allotted time limits and I’ll turn
this back over to Dr. Chole.
DR. CHOLE:
As we wrap up this morning a couple of reminders, please fill out your continuing education
forms and a comment form and I would like to remind all the members and guests about the
banquet this evening. It’s going to be in the Estate Ballroom and bring your tickets to the
banquet and pick them up if you do not already have them. So as my last act professionally as
President of the Society I would like to announce your next President, Dr. Ron Conrad, next
President of the American Otological Society.
DR. CONRAD:
I need your attention for just another minute yet. Dr. Richard Chole has been our President for
this year and will continue to be so until July 1, 2002. He has been an outstanding leader. The
program as you know has been excellent, the organization has been wonderful and he
accomplished some very important jobs this year. I’d like to present him with this plaque and
also the President’s pin. Thank you very much on behalf of this Society.
The only other thing is be sure to mark on your calendar the next meeting will be in Opry Land
in Nashville, Tennessee, May 2nd and 3rd and I hope to see you all there and I beg that you
submit excellent abstracts. Thank you very much.
IN MEMORIAM
Jean-Bernard Causse, MD
Jean Bernard Causse, MD
May 13, 1944- December 13, 2001
He was born May, 13, 1944.
Jean Bernard attended The Strasbourg Faculty of Medicine and
won first prize from the faculty there. He was intelligent, enthusiastic, happy, and emotional. He
had great difficulty speaking negatively about anyone or anything, and he always made you feel
like you were his best friend.
Jean-Bernard was a member of many organizations, received many honors and wrote 150
publications. His personal affiliations included, the American Otological Society, Otosclerosis
Study Group, American Academy of Otolaryngology, Paris Hospitals ENT Society, New York
Academy of Sciences, National Council for Noise Pollution, Politzer Society, Prosper Meniere
Society, and he was an Honorary member of the Society of Ear Research Foundation Fellows. In
1998 he was made a member of the Marquis Who’s Who in the World. He was presented with
the French Legion of Honor in1999. He received the Certificate of honor from the American
Academy of Otolaryngology. In 2000, he was awarded the New Century Award by Barons 500
Who’s Who (USA) which includes those people who have been cited as leaders for the new
century. He was made an Honorary Citizen of Memphis, Tenn.
His academic appointments included Faculty Member of the University of Nijmegen, an
Assistant at the University of Montpellier ENT Department, and Surgeon at the University of
Montpellier, France.
He was often the Guest of Honor at meetings around the globe, the guest of princes and
kings and government officials. He performed surgery in China and was a guest of the Chinese
Communist Government. All admired him. His charm and charisma could sway even the top
officials of world organizations and certainly honored everyone with whom he came to know.
He loved the phase,” If we can see far it is because of those who have come before us. We are
merely dwarfs riding on the shoulders of giants”.
He believed in the “intelligence of the heart,” and he treated all people and patients as equals.
His sense of humor kept him strong. He loved telling jokes, he was always spinning a good
yarn, and best of all, he was always ready to make fun of himself first, and his friends second.
He was a master surgeon and loved to entertain people in his clinic.
Everyone around the
world admired his great surgical skills and his patients came from everywhere. He was the only
surgeon I know who made 2 videotapes of his surgery – 1 for the referring physician and 1 for
the patient’s family.
Over 350 doctors would visit him in his clinic during the course of a year, and he also
traveled and lectured extensively.
Jean Bernard was surgically booked for 2 years in advance. He was a master at stapes surgery
and correction of congenital middle ear defects; He performed about 25,000 stapes surgeries
which is more than anyone else in the world, and developed 54 different prostheses to make
surgery even more efficient with better hearing results. He could do things surgically that some
of us could not even dream of duplicating.
Perhaps the most difficult time for him was when he sold his new clinic in Colombiers. Because
he trusted everyone, he did not realize the consequences of selling the clinic without the advice
of an attorney. The result was disastrous and it hurt him deeply. Perhaps the stress of this entire
transaction began his illness. Strong to the core, he began a new clinic in Beziers in 1998 and his
patients faithfully followed him there. He struck up again, performing 5 cases a day, until he
really began to get ill in March.
His father, Jean-Rene Causse,MD., was a prominent otologist and started a large Clinic in
Beziers, France and performed 22,600 stapedectomies. He passed away 3 days before Jean
Bernard at age 93.
Jean Bernard spoke about retiring at the age of 60 to become a professional photographer, but
that was not in his cards. Many of his pictures won prizes. He fought very hard to stay alive for
nearly a year and with the help and support of his family and friends maintained as cheerful an
outlook as was possible. Primary Amyloidosis is a horrible disease with no known cure. His son
Jerome learned as much as possible about the disease so he could help find new treatments and
better inform people. He tried to obtain the experimental agent human Interleukin6 from Japan,
which may one day help treat this disease, but the drug came too late.
At first, Jean Bernard tried to keep his illness a secret, but eventually he let me spread the word
to his American friends. His wife, Isabel, son Jerome, and daughter Annabel stayed close and
took care of him during his illness. He was very happy to have the support of his family during
this difficult time.
The funeral was held in a 12th century Cathedral in Beziers, France. He loved this place and
used to take friends through its hallowed halls showing off the beautiful art inside.
An estimated 700 people came to pay their respects to this great man and 2 priests gave a
magnificent mass. One of the young priests compared Jean Bernard to John the Baptist. He was
sent to earth to do his special work, and when that time was done, God called him home. Jean
Bernard was at peace when he passed.
Although he suffered physically more than anyone
should, his mind was clear and his sense of humor never left.
He is survived by his wife Isabel and his children, Jerome and Annabel.
John Emmett poignantly said, “If this were the 16th century, he would surely be a
Knight.” But, in my book, Jean Bernard was a King among men. With that note, I wish him
good night, and God’s speed. He will forever reign in my heart as one of the greatest men on
earth and my best friend.
Dr. Causse became a Corresponding Member of the American Otological Society in
1995.
Written by Herbert Silverstein, MD
Ralph J. Caparosa, MD
Sketch drawn by Mark Caparosa
Ralph J. Caparosa, MD was born in Clairton, PA on February 8, 1924. He graduated from St.
Vincent College and Long Island College of Medicine. He completed his internship and
residency programs at Mercy Hospital in Pittsburg and served as a Captain in the U.S. Army. Dr.
Caparosa was board certified in Otolaryngology. He later limited his practice to the subspecialty
of Otology and Neurotology. He was a Clinical Professor of Otolaryngology at the University Of
Pittsburg School Of Medicine and served on the staff at Pittsburg Eye and Ear Hospital.
He also was on the staff at the Vetrans Adminstration Hospital, Mercy Hospital, Divine
Providence Hospital, Braddock Hospital, Montefiore Hospital and South Side Hospital.
He was a member of numerous professional organizations. He was recognized for his
distinguished work as a medical educator as well as his 42 year commitment to Otology and
Neurotology as a physician and surgeon. Dr. Caparosa was a kind and generous man who spent
endless hours devoted to teaching and patient care.
He received numerous teaching awards nationally and internationally for his work in 3-D
presentations of surgical anatomy of the temporal bone. He was honored for distinguished
service in educational programs by the American Academy of Otolaryngology.
Dr. Caparosa died on May 9, 2001 at Forbes Hospice in Pittsburg, PA from complications
following a cerebral hemorrhage. He is survived by his six children and 11 grandchildren.
Ralph J. Caparosa, MD became and Active member of the American Otologic Society in 1972
and a Senior Member in 1992.
This memorial was kindly supplied by Rosemary Caparosa Siegfried
NEW MEMBERS
Active
Moises A. Arriaga, MD
M. Jennifer Derebery, MD
Karen J. Doyle, MD
Joseph G. Feghali, MD
Michael McGee, MD
Terrence P. Murphy, MD
Steven M. Parnes, MD
Jay T. Rubinstein, MD
Fred F. Telischi, MD
Peter C. Weber, MD
Corresponding Member
Johannes J. Grote, MD
Honorary Member
Graeme M. Clark, MD