Download 13th International Conference on Cochlear Implants and Other

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Hearing implant treatment of patients with thalidomide associated middle and inner ear malformations
Kansy B. , Arnolds J. , HaÃ┬čkamp P. , Funk S. , Büchsenschütz K. , Munder P. , Teschendorf M. , Lang S. ,
Arweiler-Harbeck D.
University Hospital Essen, Department of Otorhinolaryngology, Head and Neck Surgery, Essen, Germany
Introduction: Thalidomide (Contergan ©, Chemie Grünenthal) is a tranquilizer, mainly prescribed in 1957-1961,
that causes heavy malformations of inner organ and extremities in unborn children, depending on the moment of
intake. In Germany, there are about 2400 patients suffering from thalidomide- related impairment, about 40%
with an affection of the inner and/ or the middle ear. For the first time, a thalidomide case with implantable
hearing aid on the one and cochlear implant on the contralateral side is presented and discussed.
Casuistic: We report about a 52 year old female patient with congenital, thalidomide associated bilateral hearing
loss. The patient was provided with hearing aids at the age of 4 years. On the right side the patient suffers from a
3rd degree dysplasia of the auricle. When presenting for the first time at the age of 51 due to an insufficient
hearing aid with progredient hearing loss on the left side, examination showed a partially developed external
auditory canal on the right side and a normal development with intact tympanic membrane on the left side.
Audiogram showed a deaf right side and a bone conduction of 40dB at 0,25kHz, decreasing at 70 dB at 2 kHz
and 60 dB at 6 kHz on the left side. Freiburger Speech discrimination test showed a hearing loss for numbers on
the left side at 90 dB, the monosyllable comprehension was 10% at 110dB. There was a strongly reduced
speech reception in free field.
Therapy: After unsuccessful optimizing of the hearing aids, surgical intervention with a vibrant soundbridge
implantation on the left side via round window coupler was performed. In the course, the patient was satisfied
with hearing and speech understanding, but still felt impaired by the missing stereoacousis. After completion of
the screening tests and patient´s profound education on possibly slow gain of speech discrimination due to the
long existing deafness, cochlea implant surgery of the right side was performed. 3 months after surgery, the
patient reports a highly improved stereoacusis and is able to make phone calls. Audiogram shows a hearing
level of 55 dB on the right side and a hearing loss for numbers of 55dB.
Conclusion: The treatment of patients with complex ear malformations is a special challenge for the treating
team. Because of very low number of data, in every case an individual solution in dialogue with the patient needs
to be found, to achieve the best possible outcome.