Title |
Progress of hearing loss in neurofibromatosis type 2: implications for future management
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Published in |
European Archives of Oto-Rhino-Laryngology, October 2014
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DOI | 10.1007/s00405-014-3317-7 |
Pubmed ID | |
Authors |
Georgios Kontorinis, Jaya Nichani, Simon R. Freeman, Scott A. Rutherford, Samantha Mills, Andrew T. King, Deborah Mawman, Sue Huson, Martin O’Driscoll, D. Gareth Evans, Simon K. W. Lloyd |
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. |
X Demographics
Geographical breakdown
Country | Count | As % |
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Unknown | 2 | 100% |
Demographic breakdown
Type | Count | As % |
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Practitioners (doctors, other healthcare professionals) | 1 | 50% |
Members of the public | 1 | 50% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
United States | 1 | 2% |
Unknown | 46 | 98% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Master | 7 | 15% |
Student > Bachelor | 7 | 15% |
Student > Doctoral Student | 7 | 15% |
Other | 5 | 11% |
Researcher | 5 | 11% |
Other | 7 | 15% |
Unknown | 9 | 19% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 18 | 38% |
Nursing and Health Professions | 6 | 13% |
Neuroscience | 5 | 11% |
Engineering | 2 | 4% |
Agricultural and Biological Sciences | 2 | 4% |
Other | 4 | 9% |
Unknown | 10 | 21% |