Audiovestibular sequelae of congenital cytomegalovirus infection in 3 children presumably representing 3 symptomatically different types of delayed endolymphatic hydrops

Three cases of congenital cytomegalovirus (CMV) infection with long-term audiovestibular sequelae are presented. Case 1 had no hearing in one ear and severe progressive hearing loss in the other ear; he showed vestibular symptoms at the age of 4.5 years. Case 2 had severe but stationary hearing loss in one ear and showed hearing impairment symptoms in the other ear at 9–13 years of age. Case 3 did not have hearing impairment symptoms, or vestibular symptoms, but was found to have severe progressive hearing loss from the age of 15 months onwards, which led to profound deafness at the age of 2 years and vestibular areflexia at or before the age of 4 years. These cases may represent 3 symptomatically different types of delayed endolabyrinthine hydrops. Type 1 (ipsilateral hydrops) incorporates vestibular symptoms only because of a lack of hearing in the offending labyrinth. Type 2 (contralateral hydrops) incorporates hearing impairment symptoms only because of a lack of vestibular function on both sides and type 3 does not incorporate hearing impairment symptoms or vestibular symptoms (other than those relating to a complete lack of function). Given the present findings, those described by Weiss and Ronis (Trans. Pa. Acad. Ophthalmol. Otolaryngol., 30 (1977) 52–54) in one case and other reported findings relating to histopathological or imaging methods in somewhat similar cases, it seems appropriate to include congenital CMV infection in the differential diagnosis of delayed endolymphatic hydrops.     

Traumatic perilymphatic fistulas in children: etiology, diagnosis and management

Post-traumatic perilymphatic fistulas have been described following ear and temporal bone injury, particularly in the setting of temporal bone fractures. However, indications for exploratory surgery in cases of trauma without temporal bone fracture are vague and not well described. We describe three children who presented with symptoms suggestive of perilymphatic fistula (PLF) without an associated temporal bone fracture: two with penetrating tympanic membrane injuries and one with blunt temporal bone trauma. All had symptoms of hearing loss and vestibular disturbance. Two of the children cooperated with ear-specific audiologic assessment, which demonstrated sensorineural hearing loss (SNHL) on the traumatized side. The third child showed audiometric evidence of a SNHL on the injured side, but due to his age, the degree of severity of the SNHL was unable to be appropriately addressed prior to the patient being surgically managed. All three children underwent exploratory surgery and were found to have bony defects in the region of the oval window. All were repaired with fascial grafts to the oval and round windows with complete resolution of vestibular symptoms. However, two of the three patients with documented post-operative audiograms suffered from persistent SNHL on the injured side. We conclude that exploratory middle ear surgery is indicated in patients suffering from blunt or penetrating temporal bone or middle ear trauma who demonstrate persistent vestibular symptoms, sensorineural hearing loss or radiographic evidence of oval window pathology. As this is a limited number of patients, a larger series may be warranted to study the actual incidence of post-traumatic PLF in the child with persistent hearing loss and vertigo after head or ear trauma.     

Middle-ear ceruminous adenoma as a rare cause of hearing loss and vertigo: case reports

Middle-ear ceruminous adenomas are rare benign neoplasms arising from the epithelium of the middle ear. Progressive hearing loss, ear fullness and tinnitus are common symptoms of this tumour; facial nerve paresis and vestibular disturbances occur very infrequently. We present two cases of middle-ear ceruminous adenomas, one showed rapid unilateral hearing loss with aural fullness, followed by purulent aural discharge and vertigo. In the second case, the disease affected an already deaf ear and the only symptom of the disease was increasing vertigo. The clinical features, intraoperative findings, and histological and radiological findings are presented. The cases are compared to those described in the literature.     

Pediatric temporal bone fractures

Temporal bone trauma are more common nowadays related to sports. We report 2 cases with temporal bone fractures in patients younger than 14 years. They were two male, 9 and 12-year-old respectively, that presented traumatism after accidental falls from lowe height. The first case had a longitudinal temporal bone fracture without hypoacusia or vertigo, whereas the second one had an oblique fracture involving the otic capsule, provoking permanent neurosensorial hearing loss, as well as vertigo and unsteadiness that resolved later, related to a vestibular hyporreflexia. Neither case had facial paresia or paralysis. CT scanning was essential to determine the importance of the trauma. Despite the otic capsule rupture, hearing loss in the second case was only significant over 2 KHz, suggesting a selective cochlear trauma. We present clinical and imaging findings, as well as correlation of audiological and vestibular alterations, reviewing the clinical presentation of temporal bone trauma in children.     

Asymmetrical hearing loss following symmetrical acute acoustic trauma

Due to diffraction, acute acoustic trauma usually causes symmetrical hearing loss. Acoustical shadow effects are relevant only at distances greater than 1 m to the sound source and frequencies greater than 1000 Hz. In case of pronounced asymmetrical hearing loss causal connection to acute acoustic trauma is unlikely. We describe two cases with pronounced asymmetrical hearing loss in spite of symmetrical acute acoustic trauma. In the first case two patients headed an exploding giant tyre in a distance of approximately 1-2 m. One of them--as expected--got a symmetrical inner ear damage. On the other hand the other patient, which stayed directly besides him, got a pronounced asymmetrical inner ear damage. In an additional case we found also asymmetrical inner ear damage after symmetrical noise exposure. In this case it was detected, that years ago the patient suffered from a menieriform attack in the now worse ear. Occult inner ear damage is discussed as a reason for the phenomenon of pronounced asymmetrical inner ear damage after symmetrical noise exposure.     

Temporal bone fracture following blunt trauma caused by a flying fish

Blunt trauma to the temporal region can cause fracture of the skull base, loss of hearing, vestibular symptoms and otorrhoea. The most common causes of blunt trauma to the ear and surrounding area are motor vehicle accidents, violent encounters, and sports-related accidents. We present an obscure case of a man who was struck in the ear by a flying fish while wading in the sea with resulting temporal bone fracture, sudden deafness, vertigo, cerebrospinal fluid otorrhoea, and pneumocephalus.     

Secondary Menière's disease

Among 93 patients presenting the typical symptoms of a Ménière's disease associating an unilateral fluctuating hearing loss of sensorineural type, tinnitus and vertiginous attacks lasting minutes to hours, 40 patients (43%) presented in their personal history a particular otologic insult in the ear which later on developed into the full Ménière's symptomatology, or a particular systemic disease with otologic manifestations. The Ménière's triad appeared in these patients six months to twenty nine years after the initial otologic or systemic lesion. Among these initial lesions were 16 cases of sudden partial or complete deafness related to viral or bacterial infection, 3 cases of sudden cochleo-vestibular deficit and 1 case of vestibular neuritis, 5 cases of temporal bone fractures and 4 cases of significant acoustic trauma, 2 cases of otosclerosis, 1 case of chronicotitis media and 1 case of severe hearing loss after otologic surgery, 5 cases of meningo-encephalitis and 2 cases of acquired syphilis. These particular lesion could be, in our opinion, the releasing factor of the inner ear dysfonction leading eventually to a secondary Ménière's syndrome.     

Non-syndromic bilateral enlarged vestibular aqueducts in two siblings

Enlarged vestibular aqueduct is one of the most frequent inner ear malformations with early manifested sensorineural hearing loss. It is often associated with Pendred syndrome. The non-syndromic familial enlarged vestibular aqueduct entity is less described with only a few cases reported in the literature. It is thought to be a varying presentation on the phenotypic spectrum of genetic mutation in the same locus of chromosome 7q31. The familial inheritance has been suspected to be autosomal recessive. In this case report, we present a patient who presented to the clinic with hearing loss after blunt head trauma with a soccer ball. Suspected enlarged vestibular aqueduct was confirmed to be bilateral on CT scan of the temporal bones. Additional inquiry into the family history revealed that her sister also had bilateral sensorineural hearing loss at an early age. Unpredictably, bilateral enlarged vestibular aqueducts were confirmed in the sister with similar imaging modality. The clinical presentation, diagnosis, and treatment strategies are reviewed along with the current literature.     

Conversion disorder in a child presenting as sudden sensorineural hearing loss

Pseudohypoacacusis is a form of conversion disorder in which a hearing loss is found in the absence of organic disease. A case of an 11-year-old boy with auditory conversion disorder after mild head trauma is reported. The child presented with unilateral hearing loss, with no accompanying vestibular symptoms and no history of otologic disease. Auditory testing revealed severe right-sided sensorineural hearing loss, however, CT scan was normal. There was a family history of conversion disorder and the child was undergoing an emotionally stressful period. It was decided to treat the child conservatively with close monitoring and a repeat audiogram 2 weeks later. One week after presentation, the child's hearing suddenly returned to normal after a second very minor head injury. It is important to consider pseudohypoacusis or conversion disorder even in cases such as trauma, when the clinical history supports a diagnosis of hearing loss.     

Labyrinthine trauma during ear surgery

Surgical trauma inflicted upon the membranous labyrinth is an uncommon, but often catastrophic, complication of chronic ear surgery. Areas most vulnerable to injury are the oval window and the lateral semicircular canal. Methods of injury include subluxation of the footplate, fracture of the lateral canal by drill or chisel, and accidental opening of a fistula. Three cases of labyrinthotomy of the lateral canal during ear surgery are presented. Two occurred while drilling, and the other was associated with a dehiscence of the bony and membranous labyrinth from a cholesteatoma. The cases are documented by pre and postoperative audiograms, vestibular function tests, and photographs. Of interest is the fact that in none of these cases was cochlear function compromised. In one case the hearing actually improved. The dictum that an accidentally opened labyrinth will automatically result in a dead ear is shown to be not necessarily true. Possible explanations for this phenomenon are discussed.     

Various causes and clinical characteristics in vertigo in children with normal eardrums

OBJECTIVE: The differential diagnosis of vertigo in children is extensive. Otitis media and middle ear effusion could be the most common causes of vertigo in children, but there are some problems in detecting the other causes for vertigo because they are one of most frequent diseases of childhood. The purpose of this study is to review the clinical characteristics and both the audiological and vestibular findings of vertigo in children with normal eardrums, who do not show otitis media or middle ear effusion, and to assist in making a differential diagnosis of vertigo. METHODS: The fifty five children (< 16 years old) with vertigo, who visited the Department of Otolaryngology, Ajou University Hospital, Suwon, South Korea between January 1995 and December 2001 were selected for this study. These excluded the patients with abnormal eardrums/tympanograms or those that did not perform questionnaires, audiological, or vestibular evaluations. They were retrospectively analyzed for clinical symptoms, vestibular functions, and differential diagnosis. RESULTS: The most common causes for vertigo in children were migraine in 17 (30.9%) and benign paroxysmal vertigo of childhood (BPVC) in 14 (25.5%). Other less frequent causes included four cases of trauma, two cases each of Meniere's disease, delayed endolymphatic hydrops, benign positional vertigo, and one case only for cerebellopontine angle tumor, seizure, acute vestibular neuritis, juvenile rheumatoid arthritis, leaving ten cases (18.2%) as unclassified. Abnormal findings were noted in 13 (23.6%) in pure tone audiogram, 3 (5.5%) in positioning test, 6 (10.9%) in bithermal caloric test, and 36 (65.5%) in rotation chair test. CONCLUSIONS: The vertigo in children with normal eardrums, who did not show otitis media or middle ear effusion, was most commonly caused by migraine and BPVC. These findings have shown to be very different from those with adult vertigo. The evaluation of vertigo in children requires a questionnaire for extensive and complete history taking, audiograms and vestibular function tests. And in selected cases, electroencephalography, hematological evaluation, imaging of the brain or temporal bone should be performed.     

Positional vertigo and cochlear implantation.

OBJECTIVE: To identify patients developing positional vertigo after cochlear implantation. STUDY DESIGN: Prospective study on a cohort of patients undergoing cochlear implantation. SETTING: Academic tertiary referral center. PATIENTS: The study included 70 consecutive patients who underwent vestibular evaluation before and after cochlear implantation. INTERVENTION: Medical record review. MAIN OUTCOME MEASURE: Recorded vestibular symptoms after cochlear implantation. Patients with positional vertigo were considered case subjects, whereas those without vestibular symptoms were considered case controls. RESULTS: Benign paroxysmal positional vertigo (BPPV) occurred in 8 patients (on the cochlear implant [CI] side in 7 patients, and in the other ear in 1). One patient had BPPV of the lateral semicircular canal on the implanted side, and 7 patients had BPPV of the posterior semicircular canal (on the same CI side in 6 patients, and on the opposite side in 1), which were detected and presented during the last examination. In 5 patients, the onset of symptoms varied from 7 to 130 days after implant activation; in 2 patients, the onset occurred before activation. CONCLUSION: Three different mechanisms are proposed for the occurrence of BPPV in patients with CI. The first focuses on the fall of bone dust particles into the cochlea during cochleostomy. In the second, the vibration caused by drilling the cochlea would be sufficient to dislodge otoconia into the labyrinth. The third hypothesis suggests dislodging of an otolith because of the electric stimulation. In our patients, conservative approaches have been used with a minimal invasive cochleostomy and without perilymph suction. Thus, the vibratory trauma affecting the cochlea during cochleostomy seems to play a fundamental role in the development of paroxysmal vertigo in patients with implant.     

Inner ear extension of vestibular schwannomas

OBJECTIVE: Inner ear extension of vestibular schwannomas (VSs) is a rare finding but has important clinical implications. This report reviews the treatment options and presents the experience of the Gruppo Otologico, Piacenza, Italy, in this field. STUDY DESIGN: Case report and literature review. METHODS: Five cases of VSs with inner ear extension were surgically removed. In all of them, the cochlea was partially or completely invaded by the lesion. RESULTS: In 4 cases, the inner ear extension was preoperatively identified on magnetic resonance imaging, and the surgical removal was planned through a transotic approach. In the last case, the cochlear invasion was not detected preoperatively, and the lesion was removed during a second surgery performed to seal a cerebrospinal fluid fistula. CONCLUSIONS: VSs with inner ear extension should be distinguished from pure intralabyrinthine schwannomas because of differences in clinical significance. Cochlear involvement is more frequent than vestibular involvement and is often accompanied by a dead ear. Dead ear caused by small VSs should alert the surgeon to the possibility of a cochlear extension. The presence of an intracochlear involvement requires the adoption of an approach that allows control of the cochlear turns, and we found the transotic approach to be the most suitable. Undetected cochlear extensions that are left in place may grow with time.     

迟发性膜迷路积水的内耳自身免疫因素研究

目的：探讨内耳自身免疫性病理因素在迟发性膜迷路积水(DEH)发病中的作用。方法：对26例(同侧性19例，对侧性7例)DEH患者行临床观察、听觉功能测试、免疫学试验；并施以药物治疗、免疫抑制剂治疗、经迷路-耳蜗神经切断术及迷路后前庭神经切断术治疗，再进行疗效分析。结果：特异性抗体阳性：同侧性4例，对侧性l例；对侧性l例特异性细胞免疫反应阳性；同侧性l例循环免疫复合物阳性。ll例药物治疗可控制眩晕发作，8例经行前庭神经切断术后眩晕消失，1例行对侧原发性聋耳鼓室成形术后眩晕消失，4例免疫抑制剂治疗有效，1例自然缓解，1例经药物、免疫治疗及内淋巴囊引流术后无效。结论：导致先期耳聋的常见病因有突发性聋、脑膜炎、麻疹、乳突手术等；DEH与原发性聋的间隔期为1～34年。DEH症状较典型梅尼埃病更难控制。内耳自身免疫性病理因素在本病发病中可能起着重要作用。     

内耳畸形聋儿人工耳蜗植入术常见类型及手术并发症

目的 探讨内耳畸形聋儿实施人工耳蜗植入术时常见的类型及并发症。方法 回顾性分析电子耳蜗植入术病历资料170例,对其中的32例双侧内耳畸形患者加以畸形类型及手术并发症总结。结果 ①人工耳蜗植入患儿内耳畸形所占比例(32/170,18.8%)明显高于其他文献报道;②32例内耳畸形中,大前庭导水管23例(占全部畸形数71.3%),大前庭导水管伴其他类型畸形者5例(并发Mondini畸形4例,并发外半规管未发育1例),Mondini畸形2例,Mondini畸形并发外半规管未发育前庭腔扩大1例,耳蜗CT影像疑似为“三叉”无法分类1例;③术中发生严重井喷3例(耳蜗CT影像疑似为“三叉”畸形、Mondini畸形并发外半规管未发育前庭腔扩大1例,及大前庭导水管并发Mondini畸形1例);④耳蜗影像疑似为“三叉”患者,术中发生严重井喷,电极植入困难,4个电极不能植入,术后听力未改善,半年后行对侧耳植入成功;⑤Mondini畸形并发外半规管未发育前庭腔扩大患儿术后半年并发脑脊液耳鼻漏、反复脑膜炎发作,术后1年行手术探查,后治愈。结论 ①人工耳蜗植入常见的内耳畸形包括,大前庭导水管综合征及其相伴发或单发的各类内耳畸形;②内耳畸形非人工耳蜗植入术的绝对禁忌证,但术中严重井喷多见,电极植入不完全多见,术后脑脊液耳鼻漏并发脑膜炎也多发生于畸形耳蜗,术前详细的影像学检查可以对各类畸形进行详细分类,并在术前对手术难度有充分的准备,可以减少相关并发症的发生。     

Assessment of vestibular ototoxicity of ear drops by recording of vestibular evoked potentials to acceleration impulses

INTRODUCTION: The cochlear ototoxicity of several ear drops is well documented in the literature, but very few studies exist on the vestibular ototoxicity of these topical drugs. GOAL OF STUDY: To develop an animal model for the assessment of the vestibular ototoxicity of ear drops. MATERIALS AND METHODS: Two animal groups, consisting of five fat sand rats (FSRs) each, underwent unilateral labyrinthectomy. Normal saline was topically applied into the middle ear cavity of rats in the first group for 7 days (control group). Rats in the second group were treated in the same way by topical gentamicin solution. Cochlear function was assessed by the recording of auditory evoked potential (ABPs) thresholds, and vestibular function was assessed by the recording of vestibular evoked potentials (VsEPs) to angular accelerations. RESULTS: In the control group, except for the amplitude of the first wave, there was no significant difference in the VsEPs recorded before and after topical application. In the gentamicin group, VsEPs could not be recorded after 7 days, and ABPs were recorded in one case only, with a threshold of 100 dB sound pressure level (SPL). CONCLUSION: VsEPs seem to be a reliable measure for evaluating the vestibular ototoxicity of topical ear drops.     

Recurrent meningitis secondary to idiopathic oval window csf leak

Bacterial meningitis remains a life-threatening infection even in the present antibiotic era; thus, any abnormality which predisposes a patient to a recurrence of this serious disease, must be identified and corrected. This report describes the history of a 12-year-old boy with a profound neurosensory hearing loss, a related absence of vestibular function and a Monclini-type of temporal bone dysplasia who developed recurrent episodes of meningitis which were due to an idiopathic cerebrospinal fluid otorrhea. Even though the meningitis was labyrinthogenic in origin, the patient did not experience the associated symptoms of hearing loss and/or vertigo since the affected inner ear was clinically unreactive. By surgically exploring the middle ear, the presence of a cerebrospinal fluid otorrhea was confirmed. The leak was observed to be coming from a defect in the stapes footplate, and it was controlled by firmly packing the inner ear vestibule with muscle. A remarkable similarity exists between the patient described above and the 15 previously reported cases of meningitis due to a spontaneous cerebrospinal fluid otorrhea. Generally, the problem occurred in young children, the average age being 6.4 years; male and female were equally afflicted. All 15 previously reported cases had a severe neurosensory hearing loss which was unilateral in 10 individuals and bilateral in the other five. In 11 of the case reports, the vestibular function was evaluated, and the labyrinth was noted to be unreactive in the affected ear. An associated congenital abnormality of the inner ear was described in 11 of the patients reviewed. Anatomically, in 13 cases, the leak was observed to be coming from the oval window area. Other affected sites included one report of a fissure of the promontory and one report of a defect in the roof of the eustachian tube. Multiple surgical procedures were required in 11 of the 15 patients in order to identify the exact source of the otorrhea and to seal it permanently. In three cases, the successful procedure was a middle ear exploration with stapedectomy and packing of the inner ear vestibule. Overall, a total of 36 operations was performed in the 15 patients reviewed. In conclusion, when the physician is confronted by a case of meningitis in a patient with a unilateral or bilateral total loss of hearing and vestibular function, the possible presence of an idiopathic cerebrospinal fluid leak should be considered, especially if radiographic studies demonstrate a temporal bone dysplasia. In these selected cases, if the etiology of the meningitis is obscure, a middle ear exploration should be performed both for diagnostic purposes as a means to ascertain definitely the presence of a leak and for therapeutic purposes to seal it effectively.     

Manifestaciones audiovestibulares en pacientes con paquimeningitis hipertrófica idiopática: Revisión sistemática de la literatura

Background and objectiveHypertrophic pachymeningitis is an infrequent inflammatory disease resulting in thickening and fibrosis of the dura mater. In most cases, the cause in unknown and is called idiopathic hypertrophic pachymeningitis (IHP). Audiovestibular symptoms are infrequent and the pathogenesis is still unclear.Materials and methodsA systematic literature review of cases with IHP and vestibular symptoms from 2000 to February 2020 was performed. PRISMA Checklist was followed and PubMED database, Web of Science and Cochrane library were searched. We report a case of an adolescent with a diagnosis of vestibular neuritis in the context of IHP attended in our clinic.ResultsWe reviewed 5 articles related to IHP and vestibular disorders. A total of 7 cases (5 women and 2 men), with ages between 27 and 68 years with IHP were found. They all had audiovestibular symptoms. In contrast to our patient, uni or bilateral neurosensorial hearing loss was reported in all cases. Furthermore, there is no other case report published describing the association between IHP and vestibular neuritis. High dose steroids improved symptoms in 85.7% of the patients.ConclusionVestibular symptoms in IHP are uncommon and the pathogenesis is still debatable. Entrapment of nerves in the internal auditory canal and secondary neuronal damage could be suspected as the main cause of hearing and vestibular loss.     

Metachronous Bilateral Vestibular Schwannomas

Bilateral vestibular schwannoma (BVS) is the hallmark of neurofibromatosis type 2 (NF2), both of them being present at diagnosis. We report four cases of metachronous BVS, a contralateral intracanalicular vestibular schwannomas (VS) being visible 2 to 13 years after resection of a unilateral VS. NF2 workup was negative except in one case where two NF2 gene mutations were found in tumor analysis. These cases raise the questions of whether the contralateral VS occurred by chance and how to manage it on the only hearing ear. Otologists should be aware of this rare eventuality for decision making of the first unilateral VS. Laryngoscope, 131:E250–E254, 2021     

Round window ultrasonic treatment of Meniere disease.

The neurosensory structures within the vestibular labyrinth of the inner ear are selectively irradiated with ultrasonic energy to alleviate the vertiginous symptoms of Meniere disease. However, correct application and coupling of the ultrasonic transducer to the inner ear is essential for maximum transmission of energy into the vestibule. The importance of assessing the results of long-term postoperative treatment is demonstrated by a change in the success rate that decreased by approximately 10% when the results obtained within the first 12 months of treatment were compared with those after four years. In this investigation, 32 patients were treated by the round window approach between 1970 and 1972, and after four years it was found that 72% of these were successful in relieving the giddiness symptoms.