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Objective: To explain the pathophysiology of the hearing loss in Ménière's disease (MD)., Background: In a previous report, we described a dramatic recovery of hearing in 12/31 patients with MD using antiviral (AV) drugs. The hearing loss in the remaining 19 patients with a longer history of MD remained unchanged or else worsened. Vertigo control was complete in the group with improved hearing but poorer in the group with greater hearing loss (and a longer MD history). Since achieving the recovery of hearing and control of the vertigo using AV drugs in these patients with a shorter history (≤2 years) of MD, we have continued to record dramatic hearing recovery in MD when patients have a shorter history of symptoms. We describe this here in 5 representative MD patients. We feel that the most likely explanation for the outcome is the removal of viral nucleic acids (NA) from the organ of Corti., Summary: A likely explanation for the sensorineural hearing loss in MD is paralysis of the cochlear amplifier function of the outer hair cells due to the toxicity ofNA. These NA are released from neurotropic viruses located in the vestibular nerve ganglion. Key Messages: (1) Viruses (Herpes family) are the cause of the symptoms in MD. (2) Hearing loss is the result of viral NA in the organ of Corti. (3) A short history of MD symptoms (<2 years) favors hearing recovery., (© 2021 S. Karger AG, Basel.)

The recurrent nature of the 3 most common vestibulopathies suggests a recurrent cause. Histopathology in temporal bones from patients with these syndromes - vestibular neuronitis (VN, n = 7), Ménière's disease (MD, n = 8) and benign paroxysmal positional vertigo (BPPV, n = 5) - shows focal degeneration of vestibular nerve axons and degenerated nearby facial nerve meatal ganglion cells. Transmission electron microscopic confirmation of intracytoplasmic viral particles in surgically excised vestibular nerves from patients with VN and MD support a viral etiology in these vestibulopathies. Antiviral treatment of these syndromes in a series of 211 patients with a 3- to 8-year follow-up resulted in complete control of vertigo in VN (88%), MD (90%) and BPPV (60%)., (Copyright © 2013 S. Karger AG, Basel.)

Objective: To demonstrate the organization of the spiral ganglion in the mammalian species., Methods: Temporal bone (TB) specimens from man (n = 2), monkey (n = 2), lion (n = 2) and cat (n = 20) were stained, decalcified and dissected according to the Sudan black B method of Rasmussen. These TB specimens were examined under a Zeiss operating microscope and photographed with a Canon 100 camera interfaced with the microscope., Results: Spiral ganglion cells occurred in clusters within Rosenthal's canal in all four species. The location of the clusters was marked by the interface between axon and dendritic bundles as well as groups of ganglion cells. In monkey and man the clusters were more separated than in lion and cat., Conclusions: These observations indicate that the spiral ganglion forms clusters of neurons within Rosenthal's canal at the basal cochlear turn in the mammals investigated here. The formation of clusters may be related to the principles of neurogenesis., (Copyright © 2011 S. Karger AG, Basel.)

Morphological and clinical evidence supports a viral neuropathy in Ménière's disease (MD). Quantitative examination of 11 sectioned temporal bones (TBs) from 8 patients with a history of MD revealed a significant loss of vestibular ganglion cells in both the endolymph hydropic (EH) and non-EH ears. Transmission electron microscopy of vestibular ganglion cells excised from a patient with MD revealed viral particles enclosed in transport vesicles. Antiviral treatment controlled vertigo in 73 of 86 patients with vestibular neuronitis (85%) and 32 of 35 patients with MD (91%)., (Copyright 2009 S. Karger AG, Basel.)

The concept that reactivation of latent neurotropic viruses (i.e. Herpesviridae group) in the vestibular ganglion is responsible for recurrent vestibulopathies is presented. A similar histopathologic degeneration of vestibular ganglion cells in vestibular neuronitis (VN), Ménière's disease and benign paroxysmal positional vertigo is presented to support this concept. The clinical response (relief of vertigo) to the administration of antiviral medication in these syndromes provides practical evidence of a viral neuropathy in patients with recurrent vertigo. Relief of vertigo after this treatment was 90% in VN, Ménière's disease and VN. The relief of positional vertigo (benign paroxysmal positional vertigo) was 66%., ((c) 2008 S. Karger AG, Basel)

Objective: To determine the effect on hearing and balance symptoms following singular neurectomy (SN) for benign paroxysmal positional vertigo (BPPV) in the ampullary recess of the posterior semicircular canal., Research Design: The charts of 242 patients with chronic disabling BPPV who were treated with SN over a 29-year period (1972-2001) were reviewed. The results on relief of BPPV and hearing function were recorded. A subset of 16 patients where the posterior ampullary recess was entered to expose the SN is described in detail with regard to an effect on hearing and balance., Results: A total of 252 SN were performed in 242 patients. Ten patients underwent bilateral SN sequentially; the remaining 232 patients had unilateral SN. The ages of the patients ranged from 21 to 86 years, with a mean at 57 years. The female:male ratio was 174:68. Complete relief of BPPV was achieved in 244 patients (96.8%), incomplete relief in 3 (1%), and no relief in 5 (2%). Sensorineural hearing loss (SNHL) occurred in 9 patients (3.7%). A subset of 16 patients in whom the ampullary recess was opened during SN ranged in age from 21 to 79 years, with a mean at 56 years. The female:male ratio was 12:4, with right and left sides divided almost equally. Relief of BPPV was achieved in all 16 patients with no loss of hearing function. Five patients complained of a fistula response postoperatively (31%). The fistula response resolved by 6 months postoperatively in all 5 patients., Conclusions: SN is effective in relief of BPPV with little risk of SNHL (3.7%). The risk of SNHL is not increased when the posterior ampullary recess must be entered in order to transect the singular nerve. A positive fistula response may be present temporarily in almost one third of these patients., (Copyright 2002 S. Karger AG, Basel)