"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.

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.

[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.

[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.

Early uneven ear input induces long-lasting differences in left-right motor function.

How asymmetries in motor behavior become established normally or atypically in mammals remains unclear. An established model for motor asymmetry that is conserved across mammals can be obtained by experimentally inducing asymmetric striatal dopamine activity. However, the factors that can cause motor asymmetries in the absence of experimental manipulations to the brain remain unknown. Here, we show that mice with inner ear dysfunction display a robust left or right rotational preference, and this motor preference reflects an atypical asymmetry in cortico-striatal neurotransmission. By unilaterally targeting striatal activity with an antagonist of extracellular signal-regulated kinase (ERK), a downstream integrator of striatal neurotransmitter signaling, we can reverse or exaggerate rotational preference in these mice. By surgically biasing vestibular failure to one ear, we can dictate the direction of motor preference, illustrating the influence of uneven vestibular failure in establishing the outward asymmetries in motor preference. The inner ear-induced striatal asymmetries identified here intersect with non-ear-induced asymmetries previously linked to lateralized motor behavior across species and suggest that aspects of left-right brain function in mammals can be ontogenetically influenced by inner ear input. Consistent with inner ear input contributing to motor asymmetry, we also show that, in humans with normal ear function, the motor-dominant hemisphere, measured as handedness, is ipsilateral to the ear with weaker vestibular input.

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.

Hypothalamic hamartomas and inner ear diverticula with X-linked stapes gusher syndrome - new associations?

X-linked stapes gusher syndrome is a genetic form of deafness with distinct radiographic features on temporal bone CT. Hypothalamic hamartoma is a congenital glioneuronal anomaly of the hypothalamus. We report a potential association between these two rare anomalies that, to our knowledge, has not been reported. Two brothers presented with sensorineural hearing loss and almost identical inner ear and hypothalamic abnormalities, consistent with a diagnosis of X-linked stapes gusher syndrome and hypothalamic hamartoma. Genetic testing revealed identical mutations in the POU3F4 gene associated with X-linked stapes gusher syndrome. Furthermore, multiple vestibular diverticula were seen in both brothers, which have also not been reported with X-linked stapes gusher syndrome. This case suggests that POU3F4 mediated X-linked stapes gusher syndrome may also lead to multiple vestibular diverticula and hypothalamic hamartoma and, therefore, brain magnetic resonance imaging (MRI) could be considered in patients presenting with these inner ear findings.

Middle-ear cholesteatoma co-existing with labyrinthine fistula and vestibular schwannoma.

BACKGROUND: Many conditions, among them vestibular schwannoma and middle ear cholesteatoma with lateral semicircular canal destruction, may be associated with asymmetrical sensorineural hearing loss (SNHL) and vertigo. However, the probability that these two distinct disease entities causing the same symptoms occur in a single patient is very low, approximately 1 per 28 billion per 1 year. METHODS: We present the case of a 40-year-old male admitted to our clinic because of chronic middle ear inflammation with concomitant tinnitus vertigo, and deafness in the right ear. The patient was diagnosed with lateral semicircular canal fistula caused by middle-ear cholesteatoma and concomitant vestibular schwannoma. Canal wall-down surgery was carried out to remove the cholesteatoma, followed by gamma knife radiosurgery for the vestibular schwannoma. RESULTS: Vertigo and tinnitus resolved within 3 days after the ear surgery, and gamma knife treatment resulted in the complete involution of the vestibular schwannoma. The patient presented with completely dry middle-ear cavity and no recurrence of the cholesteatoma was observed during a 3-year follow-up. CONCLUSION: As the hereby reported condition is very rare, the results cannot be compared with any similar report published previously. Nevertheless, based on the outcome, the treatment strategy seems to be both reasonable and effective.

Audiological outcomes in sudden sensorineural hearing loss with presumed inner ear hemorrhage.

OBJECTIVE: To explore the hearing outcomes and prognostic factors in patients with sudden sensorineural hearing loss resulting from inner ear hemorrhage. METHODS: 42 patients (22 male and 20 female) were recruited from January 2016 to December 2017. Intravenous methylprednisolone and/or intratympanic corticosteroid were used as salvage therapy. The main measures included systemic risk factors and audiometric outcomes as proposed by American Academy of Otolaryngology-Head and Neck Surgery Hearing Loss Scale. All individuals were assessed at baseline, discharge (2 weeks post-treatment) and at 1, 3 and 6 months. RESULTS: The mean ages of patients were 39.3 ±â€¯14.8 yrs. Cardiovascular disorders were seen in 19.0-33.3% of cases. Restoration of hearing and speech discrimination abilities were assessed at the first month post-treatment versus initial levels (95.5 ±â€¯15.5 vs. 109.2 ±â€¯9.6 dB, p = 0.000; and 17.6 ±â€¯24.4 vs. 1.3 ±â€¯4.0%, p = 0.003, respectively). Word recognition scores continued to recover at month 6 (38.7 ±â€¯35.4%, p = 0.000), whereas puretone ceased to change (90.8 ±â€¯16.2 dB, p = 0.139). The final percentages of complete, partial and no recovery were 0%, 57.1% and 42.9% respectively. The prognosis was independent of accompanying systemic risk factors as analyzed in this study. Intratympanic intervention was associated with improved word recognition scores, although intravenous corticosteroid was not. CONCLUSIONS: Profound sudden sensorineural hearing loss caused by inner ear hemorrhage often has an unsatisfactory prognosis. However, this cohort did experience partial audiological recovery with delayed onset. Immediate and effective intratympanic corticosteroid may have therapeutic potential for this intractable disease.

Patients' experiences of living with superior canal dehiscence syndrome.

OBJECTIVE: The study investigated how the symptoms of superior canal dehiscence syndrome (SCDS) affected patients in their daily life, and how patients coped with the disease. DESIGN: This was a qualitative study; semi-structured interviews were performed and analysed according to the systematic text condensation method. STUDY SAMPLE: Twelve of 13 identified patients with SCDS in the county of Norrbotten, Sweden, were included in the study. RESULTS: Five main categories were created based on the patients' experiences of living with SCDS: (1) Experiencing strange symptoms: One "new" symptom was identified - mental fatigue. (2) A restricted life socially, physically and at work: All patients experienced some extent of limitation in their daily life. (3) To accept and to protect oneself: All patients had developed strategies to protect their ears from noise. (4) Misunderstood in health care: The diagnosis was sometimes delayed several years due to lack of knowledge among healthcare workers. (5) Carefully considering treatment (surgery): Symptoms were weighed against the risk of side effects. CONCLUSIONS: SCDS was rendered an invisible disability. In the present study, we identified mental fatigue as a symptom not previously considered in the literature.

Enhanced Auditory Sensitivity to Body Vibrations in Superior Canal Dehiscence Syndrome.

A key feature of superior canal dehiscence (SCD) syndrome is supranormal hearing of body sounds. The aim of the present study was to quantify this phenomenon and to ascertain whether auditory sensitivity to body vibrations can distinguish SCD patients. Hearing thresholds in response to vibration at the vertex, at the spinous process of the 7th cervical vertebra, and at the medial malleolus were tested in 10 SCD patients and 10 controls. Both patients and controls had insert earphones in both ears. The insert in the test ear was blocked while masking was presented to the other ear. Vibration in the frequency range of 125-1,000 Hz was presented to each of the 3 stimulation sites. The SCD patients were found to have significantly lower hearing thresholds compared with controls. The two study groups reacted differently with respect to frequency. The SCD patients showed an enhanced sensitivity for the lower stimulus frequencies. The difference was, however, rather independent of stimulus presentation site. The findings suggest that hearing thresholds in response to low-frequency body vibration at sites distant from the ears can distinguish SCD patients. The present findings may also support the idea that auditory sensation to body vibrations is a response related to soft tissue conduction.

Posterior semicircular canal dehiscence secondary to jugular enlargement.

Semicircular canal dehiscence is an important entity often presenting with symptoms of noise or pressure induced vertigo, autophony, aural fullness and conductive hearing loss. Due to its varied presentation and mimic of other otologic conditions its consideration is of increased importance. Within we report a case of right sided posterior semicircular canal dehiscence secondary to an enlarged and high jugular bulb. The results of this observation indicate a need for patients with auditory symptoms and high riding jugular bulbs to be evaluated for possible posterior semicircular canal dehiscence.

[Vertigo in the Emergency Department: new bedside tests]. / Szédülés vizsgálata a sürgosségi osztályon: új, ágy melletti diagnosztikai eljárások.

According to international statistics, the first examination of 25% of patients with vertigo is carried out in Emergency Departments. The most important task of the examining physician is to diagnose life threatening pathologic processes. One of the most difficult otoneurological diagnostic challange in Emergency Departments is to differentiate between dangerous posterior scale stroke presenting with isolated vertigo and the benign vestibular neuritis.These two disorders can be safely differentiated using fast, non-invasive, evidence based bedside tests which have been introduced in the past few years. 35% of stroke cases mimicking vestibular neuritis (pseudoneuritis) are misdiagnosed at the Emergency Department, and 40% of these cases develop complications. During the first 48 hours, sensitivity for stroke of the new test that is based on the malfunction of the oculomotor system is better than the diffusion-weighted cranial magnetic resonance imaging. Using special test glasses each component of the new test can be made objective and repeatable.

Superior semicircular canal dehiscence in relation to the superior petrosal sinus: a potential cause of pulsatile tinnitus.

AIM: To examine the association between superior semicircular canal dehiscence (SSCD) and pulsatile tinnitus (PT). MATERIALS AND METHODS: Two SSCD groups included 408 unilateral persistent PT patients, and 511 controls undergoing head and neck dual-phase contrast-enhanced computed tomography (DP-CECT) for reasons other than PT. The prevalence of type I (no the superior petrosal sinus running through the dehiscence) and type II (superior semicircular canal dehiscence in relation to the superior petrosal sinus) SSCD was analysed using chi-square test. RESULTS: SSCD was identified in 5.1% (21/408) of PT ears, significantly different from 2% (8/408) of non-PT ears and 0.7% (7/1022) of controls. There was no significant difference in SSCD prevalence between non-PT ears in the PT group and controls. In the PT group, 15/21 ears were type II SSCD; 6/21 ears were type I. Fifteen combined non-PT and control ears with SSCD included two type II and 13 type I SSCD. The prevalence of type II SSCD in PT ears was significantly higher than that of non-PT ears in both groups, and the prevalence of type I SSCD in PT ears was similar to that of non-PT ears in both groups. CONCLUSION: Compared with type I SSCD, there may be a causal relationship between type II SSCD and PT.

Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report.

PURPOSE: Mondini dysplasia is a rare congenital inner ear malformation that presents with abnormal cochlear development with accompanied vestibular dilation and vestibular aqueduct enlargement. This dysfunctional anatomy provides the potential for sensorineural hearing deficits, cerebrospinal fluid leaks, and severe cases of recurrent meningitis. METHODS: We present the case of a child with Mondini dysplasia who presented with unilateral hearing loss and cerebrospinal fluid (CSF) otorrhea that was surgically repaired through a combined middle fossa/transmeatal middle ear approach to alleviate any recurrence of infection and cerebrospinal fluid otorrhea. RESULTS: Postoperatively, the patient remained neurologically stable without any further CSF leakage. CSF cultures revealed a Pseudomonas aeruginosa infection, a rare occurrence within the context of Mondini dysplasia. Retrograde bacterial spread from the external ear canal into the CSF space has been theorized as the possible pathogenesis of the resulting meningitis. The patient was successfully treated with intravenous antibiotics without any neurologic complications. CONCLUSIONS: Although Mondini dysplasia is a rare malformation, the life-threatening sequelae of meningitis that can result from the dysfunctional anatomy makes it a condition that requires elevated clinical vigilance, especially when considering children with hearing loss associated with recurrent meningitis, otorrhea, or rhinorrhea.

Ossiculoplasty with hydroxyapatite bone cement: our reconstruction philosophy.

The main objective of this study is to analyze results obtained with hydroxyapatite bone cement (HABC) ossiculoplasties. This is a retrospective study of a case series. This study was conducted in an academic hospital and tertiary referral center. A total of 127 ossiculoplasties using HABC were evaluated. Ears were divided into three groups according to procedure: group 1 involved reinforcement of the incudostapedial joint with cement and reconstruction of an incus long process defect with cement. Group 2 involved partial ossicular reconstruction between the stapes and malleus handle with HABC. Group 3 was divided into two subgroups. Group 3B entailed reconstruction of the stapes with a mobile footplate (Austin-Kartush type B = group 3B) and group 3F with a fixed footplate (Austin-Kartush type F = group 3F) using a K-Helix piston (Grace Medical, Memphis, TN, USA) or a classical titanium piston (Kurz, Fuerth, Germany) glued to the incus remnant or malleus handle with cement. Anatomical and pre- and postoperative audiological results were assessed. The mean follow-up was 26 ± 14 months. Percentages of average postoperative air-bone gap ≤ 20 dB were 95, 82.5, 50 and 83.3%, and for air-bone gap ≤ 1 0 dB, 80, 50.9, 16.6 and 50% for groups 1, 2, 3B and 3F, respectively. No complications related to the cement or extrusion occurred. Hearing outcomes also remained stable over time. In our experience, ossiculoplasty with cement provides good and stable functional results, is safe, cost effective, and easy to use. HABC with or without biocompatible ossicular prostheses allows repair of different types of ossicular defects with preservation of the anatomical and physiological ossicular chain, as well as improved stability. Reconstruction of the incus long process or incudostapedial joint defect with cement is preferred over partial ossicular reconstruction.

Congenital Malformations of the Inner Ear: Case Series and Review of the Literature.

Patients with craniofacial anomalies often present to doctors due to their noticeable disfigurement and are routinely assessed by otolaryngologists for hearing evaluation. However, small percentage of craniofacial anomaly patients may present with delayed speech though they may not have initial obvious external deformation. The objective of case series is to identify the congenital inner ear malformation. The series of clinical presentation, physical examination, investigations, treatments and follow-up results were demonstrated followed by the discussion.