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Internal Auditory Canal Decompression for Hearing Maintenance in Neurofibromatosis Type 2 Patients
Daniele Bernardeschi1, Matthieu Peyre, Michael Collin
1*AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Otology, Auditory Implants and Skull Base Surgery Department, Paris, France; ‡Sorbonne Universités, UPMC Université Paris VI, Paris, France; §INSERM UMR-S 1159, Réhabilitation Chirurgicale Mini-invasive et Robotisée de l'audition, Paris, France; ¶AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Neurosurgery Department, Paris, France.
Background:
In neurofibromatosis type 2 (NF2), multiple therapeutic options are available to prevent bilateral hearing loss that significantly affects the quality of life of patients.
Objective:
To evaluate the morbidity and functional results of internal auditory canal (IAC) decompression in NF2 patients with an only hearing ear.
Methods:
Twenty-one NF2 patients operated on for IAC decompression in a 3-year period with a minimum follow-up of 1 year were included in this retrospective study. They presented unilateral deafness due to previous contralateral vestibular schwannoma removal in 16 patients or contralateral hearing loss due to the tumor in 5 patients. Hearing level was of class A (American Academy of Otolaryngology-Head and Neck Surgery classification) in 7 patients, B in 8 patients, C in 1 patient, and D in 5 patients. Pure-tone average and speech discrimination score evaluations were performed at 6 days, 1 year, and during the follow-up. Eight patients had postoperative chemotherapy.
Results:
No case of facial nerve palsy was observed. In the early postoperative period; all patients maintained the hearing class of the preoperative period. At 1-year follow-up, all but 3 patients maintained their hearing scores; at last follow-up (mean follow-up, 23 ± 8 months; range, 12-44 months), hearing classes remained stable with only 1 patient worsening from class B to C and 1 patient improving from class D to B.
Conclusion:
Decompression of IAC seems to be a useful procedure for hearing maintenance in NF2 patients, with very low morbidity. Ideal timing and association with chemotherapy should be evaluated in the future.
Abbreviations:
FN, facial nerveIAC, internal auditory canalNF2, neurofibromatosis type 2PTA, pure tone averageSDS, speech discrimination scoreVS, vestibular schwannoma.
