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      Idiopathic sudden sensorineural hearing loss: A critique on corticosteroid therapy

      , ,
      Hearing Research
      Elsevier BV

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          Abstract

          Idiopathic sudden sensorineural hearing loss (ISSNHL) is a condition affecting 5-30 per 100,000 individuals with the potential to significantly reduce one's quality of life. The true incidence of this condition is not known because it often goes undiagnosed and/or recovers within a few days. ISSNHL is defined as a ≥30 dB loss of hearing over 3 consecutive audiometric octaves within 3 days with no known cause. The disorder is typically unilateral and most of the cases spontaneously recover to functional hearing within 30 days. High frequency losses, ageing, and vertigo are associated with a poorer prognosis. Multiple causes of ISSNHL have been postulated and the most common are vascular obstruction, viral infection, or labyrinthine membrane breaks. Corticosteroids are the standard treatment option but this practice is not without opposition. Post mortem analyses of temporal bones of ISSNHL cases have been inconclusive. This report analyzed ISSNHL studies administering corticosteroids that met strict inclusion criteria and identified a number of methodologic shortcomings that compromise the interpretation of results. We discuss the issues and conclude that the data do not support present treatment practices. The current status on ISSNHL calls for a multi-institutional, randomized, double-blind trial with validated outcome measures to provide science-based treatment guidance.

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          Tinnitus with a normal audiogram: physiological evidence for hidden hearing loss and computational model.

          Ever since Pliny the Elder coined the term tinnitus, the perception of sound in the absence of an external sound source has remained enigmatic. Traditional theories assume that tinnitus is triggered by cochlear damage, but many tinnitus patients present with a normal audiogram, i.e., with no direct signs of cochlear damage. Here, we report that in human subjects with tinnitus and a normal audiogram, auditory brainstem responses show a significantly reduced amplitude of the wave I potential (generated by primary auditory nerve fibers) but normal amplitudes of the more centrally generated wave V. This provides direct physiological evidence of "hidden hearing loss" that manifests as reduced neural output from the cochlea, and consequent renormalization of neuronal response magnitude within the brainstem. Employing an established computational model, we demonstrate how tinnitus could arise from a homeostatic response of neurons in the central auditory system to reduced auditory nerve input in the absence of elevated hearing thresholds.
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            Cochlear synaptopathy in acquired sensorineural hearing loss: Manifestations and mechanisms.

            Common causes of hearing loss in humans - exposure to loud noise or ototoxic drugs and aging - often damage sensory hair cells, reflected as elevated thresholds on the clinical audiogram. Recent studies in animal models suggest, however, that well before this overt hearing loss can be seen, a more insidious, but likely more common, process is taking place that permanently interrupts synaptic communication between sensory inner hair cells and subsets of cochlear nerve fibers. The silencing of affected neurons alters auditory information processing, whether accompanied by threshold elevations or not, and is a likely contributor to a variety of perceptual abnormalities, including speech-in-noise difficulties, tinnitus and hyperacusis. Work described here will review structural and functional manifestations of this cochlear synaptopathy and will consider possible mechanisms underlying its appearance and progression in ears with and without traditional 'hearing loss' arising from several common causes in humans.
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              Acceleration of age-related hearing loss by early noise exposure: evidence of a misspent youth.

              Age-related and noise-induced hearing losses in humans are multifactorial, with contributions from, and potential interactions among, numerous variables that can shape final outcome. A recent retrospective clinical study suggests an age-noise interaction that exacerbates age-related hearing loss in previously noise-damaged ears (Gates et al., 2000). Here, we address the issue in an animal model by comparing noise-induced and age-related hearing loss (NIHL; AHL) in groups of CBA/CaJ mice exposed identically (8-16 kHz noise band at 100 dB sound pressure level for 2 h) but at different ages (4-124 weeks) and held with unexposed cohorts for different postexposure times (2-96 weeks). When evaluated 2 weeks after exposure, maximum threshold shifts in young-exposed animals (4-8 weeks) were 40-50 dB; older-exposed animals (> or =16 weeks) showed essentially no shift at the same postexposure time. However, when held for long postexposure times, animals with previous exposure demonstrated AHL and histopathology fundamentally unlike unexposed, aging animals or old-exposed animals held for 2 weeks only. Specifically, they showed substantial, ongoing deterioration of cochlear neural responses, without additional change in preneural responses, and corresponding histologic evidence of primary neural degeneration throughout the cochlea. This was true particularly for young-exposed animals; however, delayed neuropathy was observed in all noise-exposed animals held 96 weeks after exposure, even those that showed no NIHL 2 weeks after exposure. Data suggest that pathologic but sublethal changes initiated by early noise exposure render the inner ears significantly more vulnerable to aging.
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                Author and article information

                Contributors
                (View ORCID Profile)
                Journal
                Hearing Research
                Hearing Research
                Elsevier BV
                03785955
                September 2022
                September 2022
                : 422
                : 108565
                Article
                10.1016/j.heares.2022.108565
                35816890
                7a50de71-4727-4aba-9fca-420b6d3bd410
                © 2022

                https://www.elsevier.com/tdm/userlicense/1.0/

                https://doi.org/10.15223/policy-017

                https://doi.org/10.15223/policy-037

                https://doi.org/10.15223/policy-012

                https://doi.org/10.15223/policy-029

                https://doi.org/10.15223/policy-004

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