Auditory Brainstem Implantation in Neurofibromatosis Type 2: Experience From the Manchester Programme

Richard Thomas Ramsden, Simon Richard Mackenzie Freeman, Simon Kingsley Wickham Lloyd, Andrew King, Xin Shi, Charlotte Lucy Ward, Susan Mary Huson, Deborah Jane Mawman, Martin Paul O'Driscoll, Dafydd Gareth Evans, Scott Alexander Rutherford, Manchester Neurofibromatosis Type 2 Service

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OBJECTIVE: To describe the experience of auditory brainstem implantation (ABI) in patients with Neurofibromatosis type 2 (NF2).

STUDY DESIGN: Retrospective case review.

SETTING: Tertiary referral centre.

PATIENTS: Implanted with a Cochlear ABI22 or ABI24M between 1994 and 2009 because of NF2 disease.

INTERVENTION(S): Rehabilitative.

MAIN OUTCOME MEASURE(S): Surgical complication rate; audiological outcomes.

RESULTS: There were 50 primary ABI insertions in 49 patients, including 16 inserted at the time of first side tumor removal as a sleeper, and two revision repositionings which failed to improve outcome. Postoperatively three patients had cerebrospinal fluid leaks which did not require reoperation, one patient had meningitis, and eleven patients suffered either temporary or permanent lower cranial nerve dysfunction. Twenty-nine patients became full time users; a further 12 patients became non-users. Three patients died while their device was inactive. Five patients retain serviceable contralateral hearing. Audiological open set testing of users showed means of: environmental sounds discrimination 51%; phoneme discrimination: with ABI alone 22%/lip reading (LR) 45%/ABI with LR 65%; sentence testing: with ABI alone 13%/LR 19%/ABI with LR 54%.

CONCLUSIONS: The majority of patients with NF2 implanted with an ABI find the device a useful aid to communication in conjunction with LR and in recognizing common environmental sounds. A small proportion gain open set discrimination. Almost a third of patients may end up as non-users. There is probably an increased risk of postoperative lower cranial nerve dysfunction so careful preoperative assessment is advised.


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