Hearing optimisation in neurofibromatosis type 2: A systematic review

Lloyd, Simon; King, Andrew; Rutherford, Scott A.; Hammerbeck-Ward, Charlotte; Freeman, Simon; Mawman, Deborah; O’Driscoll, Martin; Evans, D. Gareth

doi:10.1111/coa.12882

Cited by 38 publications

(61 citation statements)

References 79 publications

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“…Moreover, although more widely available, PTA is not as useful in assessing hearing in NF2 as ODS, and decibel rate of loss of hearing may not be helpful to patients. Furthermore, changes in treatment in recent years, such the introduction of bevacizumab, which may better preserve hearing compared with other modalities, may mean that historical data may not be accurate when applied to newly diagnosed patients being offered currently available treatment regimens. It should be noted that all patients were included in this study regardless of whether they had undergone treatment intervention.…”

Section: Discussionmentioning

confidence: 99%

“…Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disorder with a prevalence of 1 in 60,000, which is characterized by the development of multiple benign tumors of the central and peripheral nervous system, polyneuropathy, as well as cutaneous and ocular abnormalities. Bilateral vestibular schwannomas (VS) are the hallmark of NF2 occurring in over 95% of NF2 patients, and gradual VS growth or interventions for their treatment can lead to significant hearing loss, which is often bilateral and profound . Because hearing loss significantly impacts on the quality of life for patients with NF2, hearing preservation is a key aim when considering patient management decisions.…”

Section: Introductionmentioning

confidence: 99%

“…Because hearing loss significantly impacts on the quality of life for patients with NF2, hearing preservation is a key aim when considering patient management decisions. Despite this, increasing tumor burden may necessitate treatment interventions at the cost of hearing . In patients with NF2 whose vestibular schwannomas are conservatively managed, hearing loss is usually gradual, although sudden sensorineural hearing loss has also been observed .…”

Section: Introductionmentioning

confidence: 99%

“…In addition, the rate of hearing loss in NF2 does not consistently correlate with radiological data measuring VS growth . Studies of conservatively managed tumors in NF2 13 report on more mildly affected cohorts, with the mean growth rates of conservatively managed VS being significantly slower when compared to growth rates of VS that are excised . Other potential methodological issues in previous longitudinal studies relate to relatively short follow‐up times and the eligibility criteria of patients biasing toward certain age ranges …”

Section: Introductionmentioning

confidence: 99%

“…Bilateral vestibular schwannomas (VS) are the hallmark of NF2 occurring in over 95% of NF2 patients, and gradual VS growth or interventions for their treatment can lead to significant hearing loss, which is often bilateral and profound. 2 Because hearing loss significantly impacts on the quality of life for patients with NF2, [3][4][5] hearing preservation is a key aim when considering patient management decisions. Despite this, increasing tumor burden may necessitate treatment interventions at the cost of hearing.…”

Section: Introductionmentioning

confidence: 99%

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Progression of hearing loss in neurofibromatosis type 2 according to genetic severity

et al. 2018

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Objectives/Hypothesis This study set out to describe the progression of hearing loss in patients with neurofibromatosis type 2 (NF2), treated in a quaternary multidisciplinary clinic. It also aimed to compare hearing loss across patients grouped according to a known genetic severity score to explore its utility for prognostication. Study Design Retrospective cohort study. Methods We conducted a study of 147 patients with confirmed NF2 diagnosis for a mean observational period of 10 years. Pure‐tone average (PTA), optimum discriminations scores (ODS), and genotype data were collected. Patients were classified according to hearing class (American Academy of Otolaryngology), their candidacy for auditory implantation (UK National NF2 consensus) and grouped by genetic severity as: 1 = tissue mosaic, 2A = mild classic, 2B = moderate classic, and 3 = severe. Survival analysis investigated the effect of genetic severity on the age of loss of serviceable hearing. Results Genetic severity was a significant predictor of hearing outcomes such as ODS, hearing classification, and maximum annual PTA deterioration. Although the overall median age of loss of serviceable hearing was 78 years, there was significant variation according to the genetic severity; the median for severe patients was 32 years compared to a median of 80 for tissue mosaic patients. Conclusions This is the first description of long‐term hearing outcomes in a clinical setting across a large heterogeneous cohort of patients with NF2. The results highlight the potential importance and benefit of considering the genetic severity score of patients when undertaking treatment decisions, as well as planning future natural history studies. Level of Evidence 2c Laryngoscope, 129:974–980, 2019

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Progression of hearing loss in neurofibromatosis type 2 according to genetic severity

et al. 2018

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Longitudinal Performance of Cochlear Implants in Neurofibromatosis Type 2

Seo,

Shen,

Ding

et al. 2023

The Laryngoscope

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ObjectiveCochlear implants (CIs) are a well‐established treatment modality for hearing loss due to neurofibromatosis type 2 (NF2). Our aim is to investigate variables that affect longitudinal performance of CIs among patients with NF2.Study DesignRetrospective review at a single academic institution consisting of patients who have received cochlear implants following hearing loss due to NF2.MethodsThe primary outcome examined was CI disuse or explantation. Associated clinical and surgical variables were analyzed using descriptive statistics. These included postoperative pure tone average (PTA) at 500, 1000, and 2000 Hz, tumor size, previous surgery, and comorbid depression.ResultsA total of 12 patients and 14 cochlear implants received at our institution from 2001 to 2022 were included. Notably, 35.7% of CIs (5 out of 14 cases) resulted in disuse or explantation. The average interval until explant was 9.4 years (range 3–14 years). In explanted CI cases, 20% had previous surgery and 80% had a diagnosis of comorbid depression as compared to 22.2% and 22.2%, respectively, in intact CI cases. Maximum tumor diameter was the only variable found to impact CI usage outcome (p = 0.028). Long‐term data showed that on average, patients benefit from 13.85 years of CI utility and a maximum PTA improvement of 45.0 ± 29.0 dB.ConclusionDespite the recurrent nature of NF2, patients continue to receive audiological benefit from cochlear implants. We found that larger tumor size may be associated with longitudinal CI failure.Level of EvidenceLevel 4 Laryngoscope, 2023

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Cochlear Implantation in Neurofibromatosis Type 2‐Related Schwannomatosis: Long‐Term Hearing Outcomes

Grenier,

Mosnier,

Ferrary

et al. 2024

Otolaryngol.--head neck surg.

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ObjectiveTo evaluate long‐term hearing outcomes following cochlear implantation in patients with neurofibromatosis type 2 and ipsilateral vestibular schwannoma.Study DesignRetrospective study.SettingTertiary general hospital.MethodsTwenty‐two patients undergoing cochlear implantation between 2004 and 2018 with at least 1 year of follow‐up were included. Patients were categorized as “users” or “nonusers” of their cochlear implant (CI). For users, speech perception (disyllabic words) without lip‐reading was assessed in quiet conditions 1‐year postimplantation, and annually thereafter. CI users were classified into 2 groups on the basis of speech intelligibility (≥40% or <40%). Demographic data, treatment options, and tumor size were also recorded.ResultsOne year after implantation, 16 (73%) patients used their CI daily. Twelve of these patients had a speech intelligibility ≥40% (mean: 74 ± 21.9%). Three had a Koos stage IV tumor. At the last visit (mean duration of follow‐up: 6 ± 5 years), 12 of these 16 patients were still using their implant daily, and 6 had a speech intelligibility ≥40%. No predictive factors for good performance at 1 year or performance stability were identified.ConclusionNeurofibromatosis type 2 is a complex disease profoundly affecting patient quality of life, and cochlear implantation should always be considered on a case‐by‐case basis. In some individuals, cochlear implantation can provide good speech intelligibility for extended periods, even posttreatment or in cases of large tumors.

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Hearing optimisation in neurofibromatosis type 2: A systematic review

Cited by 38 publications

References 79 publications

Progression of hearing loss in neurofibromatosis type 2 according to genetic severity

Progression of hearing loss in neurofibromatosis type 2 according to genetic severity

Longitudinal Performance of Cochlear Implants in Neurofibromatosis Type 2

Cochlear Implantation in Neurofibromatosis Type 2‐Related Schwannomatosis: Long‐Term Hearing Outcomes

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