[A case report of middle ear cholesteatoma complicated with labyrinthine fistulaand delayed endolymphatic hydrops].

Delayed endolymphatic hydrops （DEH） is a rare disease that causes vertigo and is often misdiagnosed as other vertigo diseases. This article reports on a patient with vertigo who was easily misdiagnosed. The patient was a middle ear cholesteatoma complicated by labyrinthine fistula （LF）; however, his vertigo was episodic vertigo, which could not be explained solely by LF causing labyrinthitis. The possibility of endolymphatic hydrops was suspected, which was confirmed by inner ear magnetic resonance gadolinium imaging. This is the first reported case of middle ear cholesteatoma complicated by LF and DEH. The patient underwent surgical resection of the cholesteatoma and three semicircular canal obstructions at the same time. During two years postoperative follow-up, the patient did not experience a recurrence of vertigo. When diagnosing vertigo diseases, a careful history of vertigo is of utmost importance.

Presentation, Management, and Hearing Outcomes of Labyrinthine Fistula Secondary to Cholesteatoma: A Systematic Review and Meta-analysis.

OBJECTIVE: The current study systematically reviewed the literature to compare auditory outcomes of patients treated for labyrinthine fistula (LF) based on characteristics of disease and surgical management. DATABASES REVIEWED: PubMed, Scopus, Web of Science. METHODS: Original series (at least five cases) published from 2000 reporting management and hearing results of LF secondary to cholesteatoma were included. Proportion and odds-ratio (OR) meta-analyses were conducted through inverse variance random-effects models based on logit transformation. RESULTS: The prevalence of LF is estimated to be 7% (95% confidence interval [CI], 5-9%). Fistulae involving the lateral semicircular canal (90%; 95% CI, 87-93%) and larger than 2 mm (53%; 95% CI, 43-64%) were common, whereas membranous involvement was less frequent (20%; 95% CI, 12-30%). Complete removal of the cholesteatoma matrix overlying the LF was mostly applied. Bone conduction (BC) preservation was frequently achieved (81%; 95% CI, 76-85%); new-onset postoperative anacusis was rarely reported (5%; 95% CI, 4-8%). A higher chance of BC preservation was associated with sparing the perilymphatic space (OR, 4.67; 95% CI, 1.26-17.37) or membranous labyrinth (OR, 4.56; 95% CI, 2.33-8.93), exclusive lateral semicircular canal involvement (OR, 3.52; 95% CI, 1.32-9.38), smaller size (<2 mm; OR, 3.03; 95% CI, 1.24-7.40), and intravenous steroid infusion (OR, 7.87; 95% CI, 2.34-26.42). CONCLUSION: LF occurs in a significant proportion of patients with cholesteatoma. In the past two decades, complete removal of the cholesteatoma matrix followed by immediate sealing has been favored, supported by the high proportion of BC preservation. Hearing preservation depends primarily on characteristics of the LF, and specific surgical strategies should be pursued. Intraoperative and postoperative intravenous steroid infusion is recommended.

Sensorineural Hearing Loss in Sjögren's Syndrome.

Sjögren's syndrome is a chronic autoimmune disease characterized by systemic dysfunction of exocrine glands, mainly the salivary and lachrymal glands. Sjögren's syndrome consists of two forms: primary Sjögren's syndrome, which is characterized by dry eyes and dry mouth without autoimmune diseases; and secondary Sjögren's syndrome, which is characterized by symptoms associated with other autoimmune diseases, such as systemic lupus erythematosus. Disease severities vary considerably from mild glandular dryness to severe glandular involvement with numerous extraglandular and systemic features. Sensorineural hearing loss is sometimes observed in both primary and secondary Sjögren's syndrome. This review article consists of (1) Pathology of Sjögren's syndrome, (2) Clinical manifestation of Sjögren's syndrome, (3) Autoimmune inner ear disease, (4) Histoanatomical features of the inner ear, (5) Immunological characteristics of the inner ear, (6) Pathophysiology of autoimmune inner ear disease, (7) Treatment for sensorineural hearing loss in Sjögren's syndrome, and (8) Future direction. Finally, we introduce a recently developed disease model of salivary gland inflammation and discuss future expectations for the treatment of sensorineural hearing loss in Sjögren's syndrome.

[Perilymphatic fistula in inner ear treated with blood patch].

Perilymphatic fistula (PLF) is a rare condition with a pathological communication between the inner ear and usually the middle ear, causing both cochlear and vestibular symptoms. PLF is typically treated with either a conservative approach or surgically with grafting. But a new treatment has been proposed: intratympanic injection of autologous blood, blood patch. This is a case report of a 21-year-old woman who presented herself with PLF and was successfully treated with blood patch.

[A case of hearing preservation during surgical treatment of distributed fistula labyrinth in a patient with chronic purulent means otitis and cholesteatoma]. / Sluchai sokhraneniya slukha pri khirurgicheskom lechenii rasprostranennoi fistuly labirinta u bol'noi s khronicheskim gnoinym srednim otitom i kholesteatomoi.

The article provides a brief overview of the epidemiology, diagnosis, clinical manifestations and surgical methods of treatment of labyrinthine fistulas (LF) in patients with chronic suppurative otitis media (CSOM) with cholesteatoma. The efficacy of various LF treatment techniques, their complications, and the principles of prevention of cochleovestibular disorders during surgical treatment are described. The prognostic criteria for the deterioration of bone conduction thresholds during LF surgery, which include a large LF size, the opening of the membranous labyrinth, and the extent of damage to the structures of the inner ear, are presented. A clinical case of surgical treatment of widespread LF in CSOM with cholesteatoma is presented, which proves the possibility of preserving the auditory and vestibular functions while observing the stages of sanitation, manipulations on the LF and obliteration of semicircular canals defects with auto tissues. Combined surgery of such a plan must certainly be accompanied by local instillation of solutions of hormonal preparations in case of a deficiency of perilymphatic fluid in the labyrinth and postoperative antibacterial and hormonal therapy.

Assessment of Metabolic Markers and Osteoporosis in 250 Patients with Superior Semicircular Canal Dehiscence Treated With Middle Fossa Craniotomy.

BACKGROUND: Superior semicircular canal dehiscence (SSCD) is caused by bony defects in the osseous shell of the arcuate eminence separating the labyrinth and the intracranial space. This pathologic third window causes hydroacoustic transmission resulting in debilitating symptoms. We examine the pathophysiologic association between metabolic markers, previous medical history, and SSCD symptoms before and after middle fossa craniotomy (MFC) treatment. METHODS: This study was conducted between March 2011 and September 2020 with patients with SSCD who underwent MFC. We used a Fisher test to compare variables, including bilateral SSCD, second surgery, ear anomaly, osteoporosis, arthritis, vitamin D, and preoperative/postoperative symptoms, and others. Point-biserial correlation analysis was performed to test correlations between continuous variables and categorical variables. RESULTS: A total of 250 patients with SSCD underwent MFC repair. There was significant postoperative resolution in all symptoms (P < 0.0001). Laboratory 25-hydroxyvitamin D values correlated with preoperative aural fullness (rpb= 0.29; P = 0.03), and preoperative disequilibrium (rpb= -0.32; P = 0.02). Serum calcium values correlated with preoperative hearing loss (rpb= 0.16; P = 0.02). Osteoporosis history (n = 16; 6%) was more prevalent in female patients (P = 0.0001), associated with higher levels of preoperative hearing loss (odds ratio, 4.56; P = 0.02) and higher postoperative hearing loss resolution (odds ratio, 2.89; P = 0.0509). CONCLUSIONS: Certain metabolic markers may predict SSCD presentation before and after surgery. Previous history of osteoporosis, autoimmune conditions, or arthritis may play a role in SSCD pathophysiology and can help predict clinical outcomes. Future evaluation should take metabolic laboratory values and acquire an exact medical history.

[Improvement of cochleovestibular function in chronic non-inflammatory perilymphatic fistulas of the labyrinth windows: cases from practice]. / Vozmozhnosti vosstanovleniya slukha pri khronicheskikh nevospalitel'nykh perilimfaticheskikh fistulakh okon labirinta: sluchai iz praktiki.

After a brief discussion of the problem of idiopathic sensorineural hearing loss and perilymphatic fistulas of the labyrinth windows, audiometric tests are identified that are informative for the preoperative diagnosis of the latter. Taking into account the own results of surgical treatment, attention is drawn to the feasibility of a wider use of exploratory tympanotomy in cases of suspected occurrence of perilymphatic fistulas of the labyrinth windows.

"Sandwich technique" enables preservation of hearing and antivertiginous effect in cholesteatomatous labyrinthine fistula.

PURPOSE: The aim of the study was to assess hearing, surgical and clinical results of the treatment in patients with cholesteatomatous labyrinthine fistula (LF) focusing on the different techniques and materials used in the management. METHODS: Study group included 465 patients. Cases with LFs discovered or confirmed during surgical procedure were thoroughly analyzed. RESULTS: LFs were noted in 11.4% of all cases. Thirty-eight patients, with all follow-up data available, were included into the further analysis. Most LFs were located in the lateral semicircular canal (87%). LFs were assessed as small in 2 cases, as medium in 24 patients while 12 were described as large. Based on Dornhoffer and Milewski classification, 50% of LFs were classified as IIa, 24% as IIb, 6 LFs were very deep (type III), while 4-superficial (type I). The size and type of LF did not influence postsurgical complaints (p = 0.1070, p = 0.3187, respectively). Vertigo was less frequent in LFs treated by "sandwich technique", especially those with opened endosteum. In 30 (79%) patients, hearing improved or did not change after surgery. Hearing outcomes were significantly better in the ears operated by means of CWU technique (p = 0.0339), in LFs with intact membranous labyrinth (p = 0.0139) and when "sandwich technique" was performed (p = 0.0159). Postsurgical bone conduction thresholds levels were significantly better in LFs covered by "sandwich method" (p = 0.0440). CONCLUSION: "Sandwich technique" (temporal fascia-bone pate-temporal fascia) enables preservation of hearing as well as antivertiginous effect in patients with cholesteatomatous labyrinthine fistula.

Inner ear salivary gland choristoma extending to the middle ear with congenital profound hearing loss and facial palsy: a case report.

BACKGROUND: Salivary gland choristoma (SGCh) is a rare benign tumor reported in several unusual sites, such as the gastrointestinal tract, the optic nerve, and the internal auditory canal, but never reported in the inner ear. CASE PRESENTATION: An 8-year-old girl with a history of left profound congenital hearing loss presented to us with ipsilateral progressive severe facial nerve palsy (House-Brackmann Grade VI). The left tympanic membrane was swollen with a pulsatile tumor. Radiological investigations revealed a multilocular tumor in the inner ear extending into the middle ear and internal auditory canal (IAC). We performed a partial resection of the tumor by transmastoid approach to preserve the anatomical structure of the facial nerve. The tumor was pathologically diagnosed as SGCh. Two years after surgery, her facial function recovered to House-Brackmann Grade II and the residual tumor did not show regrowth on MRI. CONCLUSIONS: Although the natural course of this rare tumor is unknown, a partial resection is an acceptable treatment procedure when functional recovery of the facial nerve is anticipated.

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.

Salivary Gland Choristoma of the Middle Ear in a Child: A Case Report.

Salivary gland choristoma is an extremely rare middle ear pathology. We present the case of a 10-year-old girl with unilateral conductive hearing loss. Tympanotomy showed a nonspecific middle ear mass, absence of stapes, anomaly of incus, and displaced facial nerve. It was not possible to remove the mass completely. Histology confirmed salivary gland choristoma. The hearing in this case can be improved with a bone-anchored hearing aid.

Incidence of Inner Ear Disorders in Various Forms of Acute Otitis Media in ENT Practices in Germany.

OBJECTIVE: The aim of this study was to determine the incidence of inner ear involvement in various forms of acute otitis media (AOM) in ear, nose, and throat (ENT) practices in Germany. METHODS: Patients who had been diagnosed with various forms of AOM in the years 2010 to 2017 were enrolled in the study from a nationwide, representative practice database (Disease Analyzer, IQVIA). In these patients, the incidence of simultaneous or subsequent inner ear disorders (IED) was determined within 7 days and within 12 months from the date of an AOM diagnosis. RESULTS: A total of 286 186 patients with AOM were enrolled. The most frequent diagnoses were "nonsuppurative otitis media, unspecified" (47.6%) and "otitis media, unspecified" (39.0%). The diagnoses of hemorrhagic bullous myringitis (BM) or influenza-induced AOM were very rarely found in the database. The highest incidence of IED after 7 days and 12 months was found in "nonsuppurative otitis media, unspecified" (7.7% and 15.9%, respectively), followed by "otitis media, unspecified" (5.6% and 13.5%, respectively). The incidences of the most frequent IED "hearing loss, unspecified" and "sensorineural hearing loss, unspecified" increased proportionally with increasing patient age, while the rare diagnoses of "labyrinthitis" and "ototoxic hearing loss" were evenly distributed among the age groups. CONCLUSION: In ENT practices in Germany, both various forms of AOM, as well as simultaneous or subsequent IED, are mostly being coded in an unspecific way, while specific forms such as hemorrhagic BM, influenza-induced AOM, and labyrinthitis are coded very rarely. Older patients have a higher risk of IED in AOM. A visit due to AOM seems to be a regular occasion for the initial diagnosis of hearing impairment in the elderly individuals. The highest risk of IED was found in nonsuppurative AOM.

Evaluation of the Presence of Sensorineural Hearing Loss and the Relationship With Intraoperative Findings in Cholesteatoma.

We investigated whether cholesteatoma is associated with sensorineural hearing loss (SNHL) and the effects of a lateral semicircular canal (LSCC) fistula, destruction of stapes, localization of cholesteatoma, and air-bone gap (ABG) size on SNHL. The charts of 159 patients who had received surgery for unilateral cholesteatoma were examined retrospectively. In all patients, air conduction and bone conduction (BC) thresholds in both ears were measured at 500, 1000, 2000, and 4000 Hz. Differences in BC thresholds between ears with cholesteatoma and contralateral ears were calculated. Demographics, localization of cholesteatoma, presence of LSCC fistula, condition of stapes, and ABG size on the affected ear were evaluated. There were significantly greater BC thresholds in ears with cholesteatoma than in normal ears for each frequency. Comparing the average BC differences at the different cholesteatoma locations, there were significant differences between the tympanic cavity + all mastoid cell group and attic, attic + antrum, and tympanic cavity + antrum groups. The BC differences at 4000 Hz were significantly high in patients with LSCC fistula. There were no significant relationships between the condition of the stapes and BC differences at any frequencies. There were significant correlations between average ABG and BC threshold differences at all frequencies. A significant relationship was found between cholesteatoma and SNHL. Patients with advanced cholesteatoma had significantly higher levels of SNHL. The BC threshold differences increased with increases in the ABG.

Internal Auditory Canal Diverticula in Children: A Congenital Variant.

OBJECTIVES/HYPOTHESIS: Internal auditory diverticula in adults have been found to exist independent of otosclerosis, and in the presence of otosclerosis. We sought to determine the prevalence of internal auditory canal (IAC) diverticula in a pediatric cohort, to assess whether IAC diverticula are a risk factor for hearing loss, and the co-occurrence of otic capsule hypoattenuation. STUDY DESIGN: Retrospective review. METHODS: A single-site retrospective review of high-resolution temporal bones computed tomography (CT) scans including the presence and size of diverticula and hypoattenuation of the otic capsule. Demographic, imaging, and audiometric data were collected and descriptively analyzed. Bivariate analysis of collected variables was conducted. Comparisons between sides in unilateral cases were also performed. RESULTS: 16/600 (2.7%; 95% CI [2.0%, 3.4%]) were found to have IAC diverticula. Six were bilateral. Thirty-one patients (5.2%) were found to have hypoattenuation of the otic capsule. There were no coincident cases of IAC diverticulum and hypoattenuation of the otic capsule. There was no association between the presence of IAC diverticula and age (P = .13). In six patients with unilateral diverticula, pure tone average (P = .42), and word recognition (P = .27) scores were not significantly different when compared to the normal, contralateral side. CONCLUSIONS: The prevalence of IAC diverticula in children is lower than the prevalence in adults. IAC diverticula in children likely represent congenital variants of temporal bone anatomy. Similar to adult populations, there is evidence that IAC diverticula in children are likely not an independent risk factor for hearing loss. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E1683-E1687, 2021.

Positional nystagmus in middle ear cholesteatoma with labyrinthine fistula.

Chronic otitis media with cholesteatoma can cause erosion of the dense labyrinthine bone overlying the inner ear organs, and this labyrinthine fistula allows pressure or mass-induced motion of the underlying perilymphatic and endolymphatic compartment, evoking vestibular symptoms. While the mechanism of a positive fistula test, which is conducted by increasing or decreasing the external auditory canal pressure, has been well established, the mechanism underlying positional nystagmus in labyrinthine fistula has not been discussed yet. In the present study, we propose a new hypothesis accounting for positional nystagmus in labyrinthine fistula involving the lateral semicircular canal (LSCC), i.e., the change in intracranial cerebrospinal fluid pressure by position change is transmitted to the perilymphatic space, causing ampullopetal (excitatory) or ampullofugal (inhibitory) deflection of the LSCC cupula.