Management of vestibular dysfunction and hearing loss in intralabyrinthine schwannomas.

PURPOSE: Intralabyrinthine schwannomas (ILS) are rare, benign, slow-growing tumors arising from schwann cells of the cochlear or vestibular nerves within the bony labyrinth. This study provides insight into the management of this rare tumor through a large case series. MATERIALS AND METHODS: After Institutional Review Board approval, a retrospective chart review was performed of all ILS patients treated at our institution between 2007 and 2019. RESULTS: 20 patients (9 male, 11 female) with ILS were managed at our institution. The right ear was affected in 9 patients (45%) and the left in 11 (55%). Subjective hearing loss was endorsed by all 20 patients. Average pure tone average at presentation was 72 dB nHL. Nine tumors (45%) were intravestibular, 6 (30%) were intracochlear, 4 (20%) were transmodiolar and 1 (5%) was intravestibulocochlear. Hearings aids were used in 3 patients (15%), BiCROS in 2 (10%), CI in 2 (10%), and bone conduction implant in 1 (5%). Vestibular rehabilitation was pursued in 5 patients. Surgical excision was performed for one patient (5%) via translabyrinthine approach due to intractable vertigo. No patients received radiotherapy or intratympanic gentamicin injections. CONCLUSION: ILS presents a diagnostic and management challenge given the similarity of symptoms with other disorders and limited treatment options. Hearing loss may be managed on a case-by-case basis according to patient symptoms while vestibular loss may be mitigated with vestibular therapy. Surgical excision may be considered in patients with intractable vertigo, severe hearing loss with concurrent CI placement, or in other case-by-case situations.

[Bilateral vestibulopathy].

The leading symptoms of bilateral vestibulopathy (BVP) are postural imbalance and unsteadiness of gait, deficits of spatial memory and navigation. The etiology of BVP remains unclear in more than 50% of patients: in these cases neurodegeneration is assumed. Frequent known causes are ototoxicity mainly due to gentamicin, bilateral Menière's disease, autoimmune diseases, meningitis and bilateral vestibular schwannoma, as well as an association with cerebellar degeneration. The diagnosis of BVP is based on a bilaterally reduced or absent function of the vestibulo-ocular reflex (VOR). Head impulse test (HIT), video-oculography system (vHIT), crvical/ocular vestibular-evoked myogenic potentials (c/oVEMP) and dynamic visual acuity is an additional test supporting the diagnosis. There are four different subtypes of BVP depending on the affected anatomic structure and frequency range of the VOR deficit: impaired canal function in the low-and/or high-frequency VOR range only and/or otolith function only; the latter is very rare. There are four treatment options: first, detailed patient counseling to explain the cause, etiology, and consequences, as well as the course of the disease; second, daily vestibular exercises and balance training; third, if possible, treatment of the underlying cause, as in bilateral Menière's disease, meningitis, or autoimmune diseases; fourth, if possible, prevention, i.e., being very restrictive with the use of ototoxic substances, such as aminoglycosides.

Bilateral vestibulopathy.

The leading symptoms of bilateral vestibulopathy (BVP) are postural imbalance and unsteadiness of gait that worsens in darkness and on uneven ground. There are typically no symptoms while sitting or lying under static conditions. A minority of patients also have movement-induced oscillopsia, in particular while walking. The diagnosis of BVP is based on a bilaterally reduced or absent function of the vestibulo-ocular reflex (VOR). This deficit is diagnosed for the high-frequency range of the angular VOR by a bilaterally pathologic bedside head impulse test (HIT) and for the low-frequency range by a bilaterally reduced or absent caloric response. If the results of the bedside HIT are unclear, angular VOR function should be quantified by a video-oculography system (vHIT). An additional test supporting the diagnosis is dynamic visual acuity. Cervical and ocular vestibular-evoked myogenic potentials (c/oVEMP) may also be reduced or absent, indicating impaired otolith function. There are different subtypes of BVP depending on the affected anatomic structure and frequency range of the VOR deficit: impaired canal function in the low- and/or high-frequency VOR range only and/or otolith function only; the latter is very rare. The etiology of BVP remains unclear in more than 50% of patients: in these cases neurodegeneration is assumed. Frequent known causes are ototoxicity mainly due to gentamicin, bilateral Menière's disease, autoimmune diseases, meningitis and bilateral vestibular schwannoma, as well as an association with cerebellar degeneration (cerebellar ataxia, neuropathy, vestibular areflexia syndrome=CANVAS). In general, in the long term there is no improvement of vestibular function. There are four treatment options: first, detailed patient counseling to explain the cause, etiology, and consequences, as well as the course of the disease; second, daily vestibular exercises and balance training; third, if possible, treatment of the underlying cause, as in bilateral Menière's disease, meningitis, or autoimmune diseases; fourth, if possible, prevention, i.e., being very restrictive with the use of ototoxic substances, such as aminoglycosides. In the future vestibular implants may also be an option.

Head position and increased head velocity to optimize video head impulse test sensitivity.

This study investigated the effects of head position on gain values during video head impulse tests (vHITs). Different head positions were used for vHIT of the horizontal semicircular canals of 20 healthy controls and 18 patients with unilateral vestibular loss (UVL), with head velocities ranging from 150°/s to 200°/s. Differences in vestibulo-ocular reflex gain in the control and patient groups according to head position (0° and 30° downward pitch) were analyzed. In the unaffected control group, the 30° pitched-down position resulted in a mean gain increase of up to 1.0 in both ears (right ear: 0.85 ± 0.26 for head-up and 1.05 ± 0.12 for head-down, p = 0.004; left ear: 0.75 ± 0.18 for head-up and 0.98 ± 0.16 for head-down, p < 0.001). In patients with UVL, the mean gains on the diseased side were 0.92 ± 0.16 in the head-up position and 0.82 ± 0.2 in the head-down position, at similar head velocities (p = 0.046). The pitched-down position also increased the asymmetry between ears in patients with UVL, at the same head velocity. A 30° head-down position can increase vHIT sensitivity, which resulted in increased mean gain in unaffected people and decreased mean gain in most of the patients with UVL in this study. This method may more effectively stimulate the horizontal semicircular canal. This vHIT modification may be helpful for more precisely evaluating vestibular function, thus reducing false-negative findings.