title: Progress of hearing loss in neurofibromatosis type 2: implications for future management.
abstract: The objective of this study was to describe changes in hearing over time in patients with neurofibromatosis type 2 (NF2) treated conservatively. A retrospective case review was conducted in a tertiary referral centre. Pure tone audiometry, speech discrimination scores, serviceable hearing (American Academy of Otolaryngology class A or B) and measurement of vestibular schwannoma (VS) size on magnetic resonance imaging were evaluated in 56 patients (89 ears) with NF2 with at least one conservatively managed VS. Over a mean follow-up period of 7 years (range 0.8-21 years) pure tone average thresholds increased gradually with a mean annual rate of 1.3 dB for the right ear (p = 0.0003) and 2 dB for the left ear (p = 0.0009). Speech discrimination scores dropped with an average annual rate of 1.3 and 0.34% in the right and left ear, respectively. Patients maintained serviceable hearing for an average of 7.6 years (range 2.7-19.3 years). The average annual VS growth was 0.4 mm without any correlation with hearing loss. There was a correlation between patients' age and pure tone threshold increase (p < 0.05 for both ears). In this selected population of patients with NF2, hearing threshold increases were very slow. In NF2 patients with indolently behaving tumours, serviceable hearing can be maintained for a significant length of time, making conservative management an attractive option.
authors.affiliation: University Department of Otolaryngology, Central Manchester University Hospitals NHS Foundation Trust, Manchester, M13 9PL, UK. gkontorinis@gmail.com.|Department of Otolaryngology, Institute of Neurosciences, Southern Teaching Hospital, Glasgow, UK. gkontorinis@gmail.com.
authors.forename: Georgios
authors.initials: G
authors.lastname: Kontorinis
authors.affiliation: University Department of Otolaryngology, Central Manchester University Hospitals NHS Foundation Trust, Manchester, M13 9PL, UK.
authors.forename: Jaya
authors.initials: J
authors.lastname: Nichani
authors.affiliation: University Department of Otolaryngology, Central Manchester University Hospitals NHS Foundation Trust, Manchester, M13 9PL, UK.
authors.forename: Simon R
authors.initials: SR
authors.lastname: Freeman
authors.affiliation: Department of Neurosurgery, Salford Royal NHS Foundation Trust, Manchester, UK.
authors.forename: Scott A
authors.initials: SA
authors.lastname: Rutherford
authors.affiliation: Department of Neuroradiology, Salford Royal NHS Foundation Trust, Manchester, UK.
authors.forename: Samantha
authors.initials: S
authors.lastname: Mills
authors.affiliation: Department of Neurosurgery, Salford Royal NHS Foundation Trust, Manchester, UK.
authors.forename: Andrew T
authors.initials: AT
authors.lastname: King
authors.affiliation: University Department of Otolaryngology, Central Manchester University Hospitals NHS Foundation Trust, Manchester, M13 9PL, UK.
authors.forename: Deborah
authors.initials: D
authors.lastname: Mawman
authors.affiliation: Department of Genetics Medicine, Central Manchester University Hospitals NHS Foundation Trust, Manchester, M13 9PL, UK.
authors.forename: Sue
authors.initials: S
authors.lastname: Huson
authors.affiliation: University Department of Otolaryngology, Central Manchester University Hospitals NHS Foundation Trust, Manchester, M13 9PL, UK.
authors.forename: Martin
authors.initials: M
authors.lastname: O'Driscoll
authors.affiliation: Department of Genetics Medicine, Central Manchester University Hospitals NHS Foundation Trust, Manchester, M13 9PL, UK.
authors.forename: D
authors.initials: D
authors.lastname: Gareth Evans
authors.affiliation: University Department of Otolaryngology, Central Manchester University Hospitals NHS Foundation Trust, Manchester, M13 9PL, UK.|School of Cancer and Enabling Services, University of Manchester, Manchester, M13 9PL, UK.
authors.forename: Simon K W
authors.initials: SK
authors.lastname: Lloyd
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001301:Audiometry, Pure-Tone; D002648:Child; D018450:Disease Progression; D005260:Female; D034381:Hearing Loss / Q000209:etiology* / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016518:Neurofibromatosis 2 / Q000150:complications* / Q000473:pathology* / Q000628:therapy; D012189:Retrospective Studies; D013997:Time Factors; D055815:Young Adult
keywords: Conservative treatment; Deafness; Neurofibromatosis type 2; Tumour size; Vestibular schwannoma; Watch wait rescan protocol