Does Location of Intralabyrinthine Vestibular Schwannoma Determine Objective and Subjective Vestibular Function?

BACKGROUND AND OBJECTIVE: The incidence of intralabyrinthine schwannomas is increasing, and a growing attention is given to the detrimental effects on hearing function. On the contrary, the vestibular profile of intralabyrinthine vestibular schwannomas (VSs) is still not well understood. We aimed to investigate and report the observed relationships between the intralabyrinthine location of the schwannomas and objective and subjective vestibular profile of the patients. METHODS: Retrospective cohort study of 20 consecutive individuals with sporadic intralabyrinthine schwannomas and grouped according to the intralabyrinthine location of the schwannomas. Vestibular testing consisted of the video head impulse test of all three semicircular canals, the caloric test, cervical and ocular vestibular evoked myogenic potentials, and the dizziness handicap inventory. A nonparametric unpaired t test was performed to compare groups, and Fisher's exact test was used for categorical data. RESULTS: The median video head impulse test gains (lateral, anterior, posterior) were 0.40, 0.50, and 0.75 for intravestibular schwannomas and 0.93, 1.52, and 0.91 for intracochlear schwannomas ( p = 0.0001, p = 0.009, p = 0.33), respectively. Caloric unilateral weakness had a median of 100% for intravestibular schwannomas and 14% for intracochlear schwannomas ( p = 0.0001). The mean dizziness handicap inventory was 21 for intravestibular schwannomas and 1 for cochlear schwannomas ( p = 0.02). There were no significant differences in vestibular evoked myogenic potentials according to intralabyrinthine location. CONCLUSION: By both objective and subjective measures, intralabyrinthine schwannomas with an intravestibular component has significantly worse vestibular function than schwannomas with purely cochlear involvement.

Transmastoid Surgery for Superior Canal Dehiscence: Prospective Longitudinal Objective and Patient-Reported Audiovestibular Outcomes.

OBJECTIVE: We assess symptom control, reversal and stability of objective third-window indicators, and hearing and balance function after transmastoid superior canal dehiscence plugging. To do this, we combine patient self-assessment questionnaires with auditory and vestibular test data collected at a preoperative and multiple postoperative visits. STUDY DESIGN: Prospective cohort study. SETTING: Tertiary referral center. PATIENTS: Patients who underwent transmastoid plugging for superior canal dehiscence from 2012 to 2020. INTERVENTIONS: Plugging of the superior semicircular canal using the transmastoid approach. MAIN OUTCOME MEASURES: Patients were evaluated preoperatively, 3-month postoperative, and at yearly follow-ups (up to 8 yr postoperative). Self-assessment questionnaires included Autophony Index, Dizziness Handicap Inventory, Tinnitus Handicap Inventory, and Hearing Handicap Inventory. Objective tests included audiometry, cervical vestibular evoked myogenic potential, ocular vestibular evoked myogenic potential, and video head impulse test. RESULTS: There was a statistically significant improvement in Autophony Index and Hearing Handicap Inventory total scores from the preoperative evaluation to 1 year postoperative. Dizziness Handicap Inventory and Tinnitus Handicap Inventory total scores also improved but statistically insignificant. Cervical and ocular vestibular evoked myogenic potential amplitudes and low-frequency air-bone gap showed statically significant reversal of the third-window effect preoperative versus 1 year postoperative. Video head impulse test of the superior semicircular canal revealed cases of pathological function preoperative with postoperative recovery. Results were stable over time. CONCLUSIONS: Superior canal dehiscence repair resolves symptoms of autophony and improves hearing disability. Objective third-window indicators reverse and show long-term stability. Auditory and vestibular function is preserved postoperative, even superior semicircular canal function may be normal. Data suggest that surgical outcomes should not be evaluated until 1 year postoperative.

Tinnitus suppression in a prospective cohort of 45 cochlear implant recipients: occurrence, degree and correlates.

OBJECTIVE: To determine tinnitus prevalence and severity in a cohort of unselected first-time cochlear implant (CI) recipients whose primary motive for CI was sensorineural hearing loss (SNHL), and to evaluate the effect of CI on tinnitus after cochlear implantation. METHODS: Prospective longitudinal study of 45 adult CI recipients with moderate to profound SNHL. Patients completed the Danish version of the Tinnitus Handicap Inventory (THI) and a visual analogue scale (VAS) for tinnitus burden before implantation, 4 months after implantation and 14 months after implantation. RESULTS: The study included 45 patients, of which 29 (64%) had pre-implant tinnitus. Median THI score (IQR) significantly decreased from 20 (34) to 12 (24) at first follow-up (p < 0.05) and to 6 points (17) at second follow-up (p < 0.001). Median VAS (IQR) for tinnitus burden decreased from 33 (62) to 17 (40; p = 0.228) and 12 (27, p < 0.05) at the first and second follow-ups, respectively. Tinnitus was totally suppressed in 19% of patients, improved in 48%, remained unchanged in 19% and worsened in 6%. 2 patients reported new tinnitus. At the second follow-up, 74% of patients had slight or no tinnitus handicap, 16% had mild handicaps, 6% had moderate handicaps, and 3% had severe handicaps. High pre-implant THI and VAS scores correlated with greater decrease in THI scores over time. CONCLUSION: 64% of the patients with SNHL had pre-implant tinnitus, which was decreased 4 and 14 months after implantation. Overall, 68% of patients with tinnitus improved their tinnitus handicap after CI. Patients with higher THI and VAS scores had a larger decline and the highest benefits in terms of tinnitus handicap improvement. The study findings demonstrate that the majority of patients with moderate to profound SNHL eligible for cochlear implantation benefit from complete or partial tinnitus suppression and improved quality of life after implantation.

Impact of an Endolymphatic Sac Tumor on Subjective and Objective Audiovestibular Function.

OBJECTIVE: This study reports subjective and objective audiovestibular function in patients diagnosed with an endolymphatic sac tumor (ELST), to evaluate the clinical implication of vestibular testing in ELST patients. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center/university hospital. PATIENTS: Five patients diagnosed with unilateral ELST. INTERVENTION: Subjective and objective audiovestibular tests; pure-tone average; speech discrimination score; the Video Head Impulse Test; the cervical vestibular evoked myogenic potential test; the caloric irrigation test; the Speech, Spatial and Qualities of Hearing Scale; the Tinnitus Handicap Inventory; and the Dizziness Handicap Inventory. MAIN OUTCOME MEASURE: Findings and characteristics of both subjective and objective audiovestibular functions. RESULTS: The mean pure-tone average and the mean speech discrimination score were 49 dB HL and 68% on the tumor ear and 13 dB HL and 99% contralaterally. The mean Speech, Spatial and Qualities of Hearing Scale score was 84, and the mean Tinnitus Handicap Inventory score was 64. Caloric testing showed a mean unilateral weakness of 80%. The Video Head Impulse Test of the lateral semicircular canal revealed refixation saccades on all tumor ears and an average gain value of 0.75 on the tumor ear versus 0.94 contralaterally. No cervical vestibular evoked myogenic potentials were elicited on the tumor side, whereas all but one was elicited contralaterally. The average Dizziness Handicap Inventory score was 47. CONCLUSION: A characteristic pattern of abnormal caloric findings, refixation saccades, and normal vestibulo-ocular reflex gain alongside sensorineural hearing loss on the tumor ear was identified. Asymmetric sensorineural hearing loss in any patient should lead to objective vestibular evaluation to strengthen not only a targeted tumor surveillance strategy and the indication for imaging, but also the design of vestibular rehabilitation. In addition, vestibular findings may guide the choice of surgical approach.

Cochlear Implantation Improves Both Speech Perception and Patient-Reported Outcomes: A Prospective Follow-Up Study of Treatment Benefits among Adult Cochlear Implant Recipients.

Cochlear implantation is considered the best treatment option for patients with severe-to-profound sensorineural hearing loss for whom conventional hearing aids are insufficient. We used a repeated measures longitudinal approach to evaluate speech recognition and patient-reported outcomes after cochlear implantation in an unbiased cohort of Danish adult patients in a prospective cohort study. We assessed 39 recipients before and two times after implantation using a battery of tests that included Dantale I, the Danish Hearing in Noise Test, the Nijmegen Cochlear Implant Questionnaire, and the Speech, Spatial, and Qualities of Hearing Scale. The study group improved significantly on all outcome measures following implantation. On average, Dantale I scores improved by 29 percentage points and Hearing in Noise Test scores improved by 22 percentage points. Most notably, the average Dantale score improved from 26 to 70% in the CI in quiet condition and from 12 to 42% in the cochlear implantation in noise condition when tested monaurally. Dantale demonstrated a significant positive correlation with Nijmegen Cochlear Implant Questionnaire and Speech, Spatial, and Qualities of Hearing Scale scores, while Hearing in Noise Test had no significant correlation with the patient-reported outcome measures. Patients improved significantly at 4 months and marginally improved further at 14 months, indicating that they were approaching a plateau. Our study's use of audiometric and patient-reported outcome measures provides evidence of the treatment benefits of cochlear implantation in adults, which may help physicians advise patients on treatment decisions and align treatment benefit expectations, as well as serve as a foundation for the development of new cochlear implantation selection criteria.

Vestibular Function in Pendred Syndrome: Intact High Frequency VOR and Saccular Hypersensitivity.

OBJECTIVE: Although Pendred syndrome involves anatomical abnormalities in the vestibular system and patient-perceived dizziness, the literature on vestibular function is scarce. The objective was to evaluate objective vestibular function in patients with PS using the video head impulse test (VHIT) and the cervical vestibular evoked myogenic potential (cVEMP) test, assessing the high frequency vestibulo-ocular reflex (VOR) and the saccular function, respectively. METHODS: Twenty-six subjects (52 ears) with Pendred Syndrome and severe-to-profound hearing loss (cochlear implant candidacy) were studied retrospectively. Main outcome measures were VHIT VOR gain, refixation saccades, cVEMP stimulus thresholds and amplitudes. RESULTS: In total, 4 of 52 ears (8%) had pathological VHIT outcomes (2 ears with low VHIT gain and 2 ears with saccades). The mean VHIT gain value was 0.96 (range 0.63-2.02). No patients had complete VOR loss. CVEMP responses were present in 76% of tested ears and absent in 24%. The mean cVEMP amplitude value was 192 µV. Absence of cVEMP response was associated with the presence of VHIT saccades (p = 0.038) and advanced age (rs = -0.34; p < 0.05). cVEMP amplitude was correlated with VHIT gain (rs = 0.46; p < 0.001). CONCLUSION: Despite dysmorphic vestibular anatomy and severe loss of hearing, the VHIT VOR function is normal in patients with Pendred Syndrome. However, saccular function appears to be abnormally sensitive, as documented by low cVEMP thresholds and high amplitudes, which agrees with a "third window" effect secondary to the large vestibular aqueduct.

Nordic results of cochlear implantation in adults: speech perception and patient reported outcomes.

BACKGROUND: Although cochlear implantation (CI) is widely performed in postlingually hearing-impaired adults in the Nordic countries, the literature on hearing outcomes remains scarce. Aims/objectives: To evaluate and correlate hearing outcomes after implantation. METHODS: Prospective evaluation of 40 adult first-implantation recipients pre- and post-implantation with Dantale, the Danish Hearing in Noise Test (HINT), the Nijmegen Cochlear Implant Questionnaire (NCIQ) and the Speech, Spatial and Qualities of Hearing Scale (SSQ12). RESULTS: Dantalemean increased from 50% (95% CI [41.7,58.4])) to 73% (95% CI [66.6,80.1]) and 19% (95% CI [13.3,25.1]) to 40%(95% CI [32.1,46.7]) (quiet/noise). HINTwords increased from 71% (95% CI [58.8,83.8]) to 83% (95% CI [69.8,95.4]) and 59% (95% CI [46.2,70.6]) to 73% (95% CI [63.3,82.8]) (quiet/noise). NCIQmean changed from 277 (95% CI [252.4,301.6]) to 396 (95% CI [366.7,424.7]) and SSQ12mean from 27 (95% CI [21.3,31.8]) to 48 (95% CI [39.4,55.6]). Correlations coefficients were r s = 0.39-0.74 (p < .01) between Dantale/HINT, rs = 0.78 (p < .0001) between NCIQ/SSQ12, r s = 0.41-0.59 (p < .01) between NCIQ/SSQ12 and Dantale and r s = 0.24-0.41 (ns) between NCIQ/SSQ12 and HINT. CONCLUSION: The study documents a high auditory performance and patient-perceived improvement after implantation and advocate broader awareness of implantation as treatment option in the growing population of severe-to-profound hearing-impaired adults. Correlations between hearing measures may influence future evaluation practices.