Hearing loss is one of the earliest manifestations in vestibular schwannomas with 60% of the patients having high frequency loss. Several metabolic and mechanical factors influencing the cochlea and cochlear nerve have been implicated in the hearing decline noted in patients with neurofibromatosis type 2 (NF2). Bevacizumab is being used currently at several centres and has demonstrated some effect in improving hearing in these patients. In the past few years, internal auditory canal (IAC) decompression via a middle fossa (MF) approach has been proposed as an alternative to tumour removal. This retrospective study evaluated the auditory brainstem responses (ABRs), speech discrimination scores (SDS) and hearing response in the first year after IAC decompression for small to medium vestibular schwannomas in 12 NF2 patients. The study showed that ABRs improved following decompression in four patients. This may indicate that, in some patients, compressive neuropathy may be the cause of hearing decline and could be potentially reversed with decompression. However, the authors found that this beneficial effect may not last beyond two years of follow-up. Their suggestion to follow-up NF2 patients not only with intralabyrinthine FLAIR MRI but also with SDS and ABRs are worth considering.
Follow-up of NF2 patients with ABRs, SDS and MRI
Reviewed by Gauri Mankekar
Hearing response following Internal Auditory Canal decompression in Neurofibromatosis type 2.
CONTRIBUTOR
Gauri Mankekar
Department of Otolaryngology-Head Neck Surgery, Louisiana State University Shreveport, Louisiana, USA.
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