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Journal of the American Academy of Audiology logoLink to Journal of the American Academy of Audiology
. 2025 Mar 1;36(2):125–131. doi: 10.3766/jaaa.240035

Sudden Unilateral Hearing Loss and Acute Vestibular Syndrome: A 5-Year-Old Case with Labyrinthitis

Ayşenur Aykul Yağcıoğlu *,✉, Görkem Ertuğrul †
PMCID: PMC12445271  PMID: 40175089

Abstract

Background:

Labyrinthitis, an inner ear infection affecting the membranous labyrinth, typically presents with symptoms such as dizziness, nausea, vomiting, tinnitus, and hearing loss. However, there is limited epidemiological data on this condition.

Purpose:

This case report presents the results of audiovestibular evaluation and vestibular rehabilitation regarding unilateral sudden hearing loss and acute vestibular syndrome caused by rare labyrinthitis in a pediatric patient.

Research Design:

This is a case report.

Study Sample:

The study sample included a 5-year-old girl with unilateral sudden hearing loss and acute vestibular syndrome.

Results:

The patient with labyrinthitis, diagnosed early after comprehensive audiological and vestibular evaluation, benefited from medical treatment and vestibular rehabilitation.

Conclusions:

This report objectively focuses on the importance of early medical treatment, regular audiovestibular follow-up, and vestibular rehabilitation in a pediatric patient with labyrinthitis.

Keywords: labyrinthitis, pediatric audiology, acute vestibular syndrome, sudden hearing loss, vestibular testing

Labyrinthitis is an infection of the membranous labyrinth of the inner ear. It usually manifests as dizziness, nausea, vomiting, tinnitus, and hearing loss. There are limited epidemiological data on labyrinthitis. However, it is known that the incidence increases with age (Barkwill and Arora, 2024). Infection can reach the inner ear via hematogenous means, middle ear infections, or brain infections (Wippold and Turski, 2009; Barkwill and Arora, 2024). Bacteria or viruses can cause acute inflammation of the labyrinth with local or systemic infections. Treatment approaches differ depending on the etiology and symptoms of labyrinthitis (Aryan et al, 2022). There are a limited number of case reports related to labyrinthitis in adults and even fewer in children (Jang et al, 2005; Ihara et al, 2023).

This case report presents the results of audiovestibular evaluation and vestibular rehabilitation for unilateral sudden hearing loss and acute vestibular syndrome caused by rare labyrinthitis in a pediatric patient.

CASE REPORT

A 5-year-old female patient presented to the emergency service one night in January 2021 with sudden dizziness and loss of balance. According to information received from the family, the patient could not stand independently (drop attack). There were no risk factors for hearing loss in the patient’s history (Joint Committee on Infant Hearing et al, 2000). The family reported that they did not notice the child’s hearing loss during the acute dizziness attack.

After the emergency service, the patient was first referred to the ear, nose, and throat (ENT) clinic and then to the audiology clinic for audiovestibular evaluation. According to the computed tomography (CT) report, no pathology was observed in the outer ear, mastoid cell, middle ear, cochlea, vestibule, semicircular canals (SCCs), vestibular aqueduct, internal acoustic canal widths, and facial nerve. However, radiological imaging results (magnetic resonance imaging [MRI]) showed findings in favor of labyrinthitis on the left. Steroid treatments were applied to the left ear via transtympanic injections because of sudden hearing loss in the ENT clinic. Because more than one injection was used in the treatment, a pressure equalization (PE) tube was placed in the left ear in January 2021.

AUDIOLOGICAL EVALUATION AND FOLLOW-UP

The patient presented to the ENT department for medical treatment because of sudden hearing loss in the left ear and received steroid treatment for 5 days. In the first audiological evaluation performed the day after the acute attack that occurred in January 2021, the patient had hearing thresholds no greater than 15 dB HL (decibel hearing level) in the right ear, whereas there was profound sensorineural hearing loss in the left ear (see Figure 1A). Acoustic immittance testing was performed to evaluate middle ear pressure, which revealed a type A tympanogram bilaterally. In the transient otoacoustic emission test (ILO 292 Echoport USB II ve ILO V6 Clinical OAE Software; Otodynamics), the patient passed the test in the right ear but failed in the left ear, according to the criterion of SNR > 3 dB at all frequencies. A natural sleep auditory brainstem response (Integrity V500; Vivosonic) was attempted, but no reliable results could be measured because of patient agitation.

Figure 1.

Figure 1.

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(A) The first audiogram of the case during the acute attack in 2021. (B) The case’s last audiogram was performed in 2023 after the PE tube was removed.

The last audiological evaluation was performed with an insert headphone after the PE tube was removed in October 2023. According to the last audiological assessment, the average pure tone in the right ear was less than 15 dB HL (see Figure 1B). However, in the left ear, a sloping curve indicates a high-frequency moderate sensorineural hearing loss (see Figure 1B). The speech perception threshold was 75 dB masked in the left ear and 5 dB in the right ear. Acoustic reflex thresholds were obtained at 95, 95, 90, and 100 dB HL at ipsilateral 500, 1000, 2000, and 4000 Hz for the right ear, but they were not obtained for the left ear. In addition, right lateralization in the Weber test supported sensorineural hearing loss. When the pure tone audiometry evaluation of the female patient before medical treatment (Figure 1A) and the evaluation after medical treatment (Figure 1B) were compared, improvement in hearing thresholds was observed in the left ear. Because of the evaluations, the patient was recommended a behind-the-ear hearing aid. Audiological assessment with a hearing aid in the sound field was performed during follow-up, and the patient benefited from the hearing aid.

VESTIBULAR EVALUATION AND REHABILITATION

Vestibular evaluation was completed 5 days after the acute attack began in January 2021 and before PE tube placement. Video head impulse test (vHIT; Synapsis®, Ulmer, France), cervical vestibular evoked myogenic potential (cVEMP), and ocular vestibular evoked myogenic potential (oVEMP) tests (Otometrics-ICS Chartr® EP 200; GN Otometrics A/S, Taastrup, Denmark) were performed. According to the vHIT results, the left vestibulo-ocular reflex (VOR) gains were as follows: anterior canal 0.8, lateral canal 0.41, and posterior canal 0.37. In addition, disorganized overt and covert saccades with high amplitude were observed, especially in the lateral canal (Figure 2). According to the vHIT results, the mean VOR gains of all SCCs on the right side were greater than 0.80. Air-conducted cVEMP and oVEMP tests using 500-Hz tone burst stimuli were performed on the patient to assess otolith organ function. In the patient’s cVEMP and oVEMP evaluation, although the P1-N1 wave complex could not be obtained at 126 dB SPL (decibel sound pressure level) in the left ear (Figures 3A, 4A), the P1-N1 wave complex was obtained at 116 dB SPL in the right ear. In the VHIT evaluation, the left ear lateral canal and posterior canal VOR gains were less than 0.8, and the cVEMP and oVEMP responses could not be obtained, indicating a partial vestibular hypofunction in the left ear.

Figure 2.

Figure 2.

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(A) vHIT findings from the fifth day of the acute attack before vestibular rehabilitation: left lateral and posterior SCC VOR gains were less than 0.80, and overt and covert saccades were observed, especially in the left lateral SCC. (B) vHIT findings 3 months after the acute attack after vestibular rehabilitation: although the patient’s left lateral SCC VOR gains reached greater than 0.80, overt and covert saccades disappeared as a result of vestibular recovery. However, it was observed that the patient had partial vestibular recovery in the left posterior SCC VOR gain (0.50). Ant. = anterior; L = left; Lat. = lateral; Post. = posterior; R = right.

Figure 3.

Figure 3.

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(A) cVEMP findings from the fifth day of the acute attack before vestibular rehabilitation: P13-N23 wave complex could not be obtained at 126 dB SPL in the left ear and P13-N23 wave complex obtained in the right ear. (B) cVEMP findings 3 months after the acute attack after vestibular rehabilitation. A low-amplitude P13-N23 wave complex was obtained in the left ear at 126 dB SPL.

Figure 4.

Figure 4.

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(A) oVEMP findings from the fifth day of the acute attack before vestibular rehabilitation: N1-P1 wave complex could not be obtained at 126 dB SPL in the left ear and N1-P1 wave complex obtained in the right ear. (B) oVEMP findings from 3 months after the acute attack after vestibular rehabilitation: N1-P1 wave complex could not be obtained at 126 dB SPL in the left ear and N1-P1 wave complex obtained in the right ear.

After the initial vestibular evaluation, a vestibular rehabilitation program was planned. Because it was determined that the patient had difficulty stabilizing the visual field during head movements because of decreased VOR gains, a vestibular rehabilitation program was created by giving the child modified games and gaze stabilization exercises, which were performed while sitting for 1 week and progressing to standing for the next week. The home program was designed to perform a total of six exercises, five repetitions a day, for 6 minutes, using sticks with stickers, sticks of different colors, balls, and light-up toys. The vestibular rehabilitation program was explained to the family, who were asked to keep a balance diary for each working day.

A second vestibular evaluation was performed after 2 weeks of the vestibular rehabilitation program and after PE tube placement. The family performed exercises regularly by playing games every day. In the vHIT, it was determined that the left lateral SCC VOR gains increased to a value of 0.72, but weakness continued for the left posterior SCC VOR (mean gain = 0.39). In addition, in the patient’s cVEMP and oVEMP evaluation, the P1-N1 wave complex could not be obtained in the ear at 126 dB SPL, and the patient was recommended to follow up with the same rehabilitation program. After the fourth week of vestibular rehabilitation, the vestibular rehabilitation program was updated, and exercises, including convergence, saccade, postural control, and gaze stabilization in dynamic movements, were included. A second set of vestibular rehabilitation exercises was recommended for three to five repetitions a day for 1–5 minutes, using a thick and long rope, colorful beads, picture cards, stickers, and mirror materials.

A third vestibular evaluation was conducted 3 months after the acute attack. The vHIT responses indicated vestibular recovery (see Figure 2B). In the cVEMP evaluation of the patient after vestibular rehabilitation, a low-amplitude P1-N1 (47.05-microvolt) wave complex was obtained at 126 dB SPL on the left ear (see Figure 3B), although the N1-P2 wave complex was still absent on the left side during the oVEMP test. In the vestibular evaluation performed 3 months after the acute period, a PE tube was present. In the final evaluation, despite the use of a PE tube, a cVEMP response was obtained in the left ear. It was recommended that vestibular rehabilitation be continued. However, the family did not continue vestibular rehabilitation because a significant decrease in dizziness and balance complaints was observed in the daily life of the patient.

DISCUSSION

Infection of the labyrinth often presents with dizziness, nausea, vomiting, tinnitus, and/or hearing loss (Barkwill and Arora, 2024). Viruses or bacteria can enter the inner ear, causing inflammation in the labyrinthine system or affecting the vestibular nerve. Viral labyrinthitis is the most common type of labyrinthitis, and it usually affects adults aged between 40 and 60 years (Barritt, 2014). Bacterial labyrinthitis can result from otogenic and meningitis infections. Otitis media or cholesteatoma often causes labyrinthitis of otogenic origin, resulting in unilateral symptoms. Meningitis labyrinthitis occurs more frequently in children younger than 2 years and presents with bilateral symptoms (Thompson and Amedee, 2009).

Labyrinthitis caused by otitis or meningitis infection without direct bacterial invasion of the inner ear is known as acute serous labyrinthitis. Suppurative labyrinthitis caused by direct bacterial invasion of the inner ear can cause severe irreversible hearing loss and dizziness. In the acute phase, serous labyrinthitis is not easy to distinguish from suppurative labyrinthitis. However, in serous labyrinthitis, audiovestibular function is usually preserved and symptoms are less severe than in suppurative labyrinthitis. The sudden development of vestibular symptoms and sudden loss of all hearing suggest the development of suppurative labyrinthitis (Jang et al, 2005). In labyrinthitis, it is assumed that toxins pass through the round window and affect the basal rotation of the cochlea. Therefore, hearing loss is localized at high frequencies (Jang et al, 2005). The results of this case report with unilateral sudden severe sensorineural hearing loss due to labyrinthitis are consistent with the literature. Sudden sensorineural hearing loss may lead to vestibular damage because the cochleovestibular structures share the same vasculature and are anatomically close to each other. In particular, hearing loss occurs in the basal turns of the cochlea, and high-frequency sensorineural hearing loss tends to be more related to vestibular damage because of its anatomical proximity to the vestibular organs, especially the saccular macula (Castellucci et al, 2023). In this case report, sensorineural hearing loss affecting high frequencies was observed together with vestibular dysfunction consistent with findings of labyrinthitis. Intratympanic steroid therapy is recommended for the treatment of sudden sensorineural hearing loss within 2–6 weeks of hearing loss onset. If treatment is administered with more than one injection, the patient may require placement of a PE tube (Chandrasekhar et al, 2019). In this present case, a PE tube was placed for intratympanic steroid treatment. This tube was also present during the vestibular evaluations of the patient.

The etiologies of unilateral hearing loss accompanied by vertigo include Ménière’s disease, otosyphilis, vestibular schwannoma, endolymphatic sac tumors, perilymphatic fistulas, meningitis, and labyrinthitis (Wippold and Turski, 2009). The most distinctive feature that distinguishes labyrinthitis from vestibular neuronitis is the occurrence of cochlear damage in addition to damage to the vestibular structures. Labyrinthitis is accompanied by hearing loss, and spontaneous vertigo occurs suddenly (Thompson and Amedee, 2009). Initial vertigo rarely lasts longer than 72 hours; however, balance problems and short-term vertigo attacks can persist for several weeks (Barkwill and Arora, 2024). In this case, the patient’s balance problem and drop attack were accompanied by sudden hearing loss. The patient's short-term vertigo attacks lasted for several weeks. In a case series of adult patients reported in the literature, sudden dizziness was observed in three of four patients with labyrinthitis, but spontaneous nystagmus was detected in only one of these patients. This result also indicates that nystagmus does not always occur in labyrinthitis (Ihara et al, 2023). A detailed history, ENT examination, comprehensive audiovestibular evaluation, and radiological imaging are required for differential diagnosis (Bruzzone et al, 2004; Wippold and Turski, 2009). A definitive diagnosis is made based on radiological findings (CT, MRI). Increased resolution and the application of special MRI sequences that enhance intralabyrinthine fluids allow for a more detailed analysis of labyrinthine structures and pathology (Bruzzone et al, 2004). In this case, detailed anamnesis, ENT examination, comprehensive audiological evaluations, and vestibular evaluation findings including VEMP and VHIT measurements were cross-checked with MRI results, and a finding in favor of labyrinthitis in the left ear was reported. At the same time, the combination of oVEMP and cVEMP is an objective tool to evaluate vestibular otolithic end-organ function during dynamic functional recovery in sudden sensorineural hearing loss, and VEMP recovery can predict auditory recovery (Hu et al, 2020). In addition to audiological evaluations in patients with sudden hearing loss, the evaluation of VEMP tests has also been valuable for hearing follow-up.

Sudden, unilateral complete loss of vestibular function can cause many symptoms, including vertigo, nausea, rapid eye movements (nystagmus), oscillopsia, falling on the affected side, gait ataxia, postural instability, and impaired perception of body orientation and movement. In such patients, most symptoms usually decrease within the first few days and disappear within a few weeks (Curthoys and Halmagyi, 2014). In this case report, a pediatric patient with sudden unilateral loss of vestibular function experienced symptoms, such as gait ataxia, and experienced improvement in her symptoms with early vestibular rehabilitation. Vestibular rehabilitation therapy is an exercise-based treatment that increases gaze stability, improves postural stability, and facilitates sensory integration for patients (Meng et al, 2023). Through vestibular rehabilitation, vestibular compensation alleviates the feeling of dizziness and imbalance in patients with vestibular dysfunction (Singh et al, 2022). Acute unilateral vestibular loss is sensitive to early-initiated vestibular rehabilitation. The period immediately after disease onset is highly sensitive to somatosensory and visual input and stimuli. Therefore, if rehabilitation is started as soon as possible, the recovery process will be faster (Deveze et al, 2014). We believe that vestibular rehabilitation accelerated the vestibular compensation process in this case. Vestibular recovery was observed in vHIT responses with vestibular rehabilitation. However, recovery in the saccule was achieved later than in the SCCs. In a study examining the prognosis of vestibular dysfunction in idiopathic sudden sensorineural hearing loss with vertigo, recovery rates of the vestibular apparatus were examined. In support of the findings of our study, it was reported that the otolith organs were at higher risk of being affected, showed worse recovery than the SCCs, and the lowest recovery rate was in the utriculus. This situation was explained by the proximal anatomical correlation of the cochlea and vestibule on the cochlea and SCC (Hao et al, 2023).

Halmagyi et al (1994) reported that VEMP responses were absent in conductive hearing loss (CHL) patients with ABG > 20 dB (Halmagyi et al, 1994). VEMP responses are believed to be related to middle ear function. In a study conducted on patients with tympanic membrane (TM) perforation, patients with less than 50 percent TM perforation were categorized into the small or medium group, and those with more than 50 percent were placed in the large group. No significant change in VEMP parameters was observed in small- and medium-perforation groups, whereas a noticeable reduction in p13 latency occurred in patients with large perforations. In the study, they observed no significant change in latency or asymmetry rate with paper patch application, regardless of TM perforation size (Lee et al, 2014). Similarly, the small- to medium-sized perforation by the PE tube did not impact the cVEMP response in this case report. The obtained cVEMP responses in the left ear despite the use of a PE tube at the final evaluation may be because of vestibular recovery after vestibular rehabilitation, independent of the PE tube. In another study, VEMP responses were compared before and after simulation in patients with CHL created by closing the external ear canal with a soundproof earplug. The simulated oVEMP and cVEMP responses constituting CHL showed decreased response rates, increased thresholds, and lower amplitudes than the nonsimulated responses, and the differences were statistically significant. In addition, significantly prolonged N1 latencies were detected in oVEMP responses. It has been reported that after simulated CHL, response rates for both oVEMP and cVEMP decreased significantly from 100 to 38.1 percent and from 100 to 61.9 percent, respectively (Han et al, 2016). In the present case report, the fact that cVEMP was obtained after vestibular rehabilitation but oVEMP responses could not be obtained may be related to CHL caused by perforation after PE tube application. However, there is not enough evidence to support this claim in the literature. Considering the preferred medical treatment methods of ENT physicians, the most suitable vestibular assessment approach for each patient should be chosen by audiologists. In patients with CHL, using bone-conducted VEMPs may produce more accurate and reliable results. The limitation of this study is that bone-conducted VEMP was not used for vestibular assessment.

CONCLUSION

In this case report, the authors have indicated the importance of early medical treatment and regular audiovestibular follow-up, and vestibular rehabilitation was objectively observed in a pediatric patient with labyrinthitis. It should be kept in mind that a complete audiovestibular assessment and early vestibular rehabilitation program are very important in patients with sudden hearing loss and acute vestibular syndrome.

Abbreviations

CHL

conductive hearing loss

CT

computed tomography

cVEMP

cervical vestibular evoked myogenic potential

dB HL

decibel hearing level

dB SPL

decibel sound pressure level

ENT

ear, nose, and throat

MRI

magnetic resonance imaging

oVEMP

ocular vestibular evoked myogenic potential

PE

pressure equalization

SCC

semicircular canal

TEOAE

transient otoacoustic emission

TM

tympanic membrane

VEMP

vestibular evoked myogenic potential

vHIT

video head impulse test

VOR

vestibulo-ocular reflex

Footnotes

Any mention of a product, service, or procedure in the Journal of the American Academy of Audiology does not constitute an endorsement of the product, service, or procedure by the American Academy of Audiology.

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