Absent pyramidal eminence and stapedial tendon associated with congenital stapes footplate fixation: Intraoperative and radiographic findings.

OBJECTIVE: Report an association between congenital stapes footplate fixation (CSFF) and radiological absence of the pyramidal eminence and stapedial tendon. PATIENTS: Children and adults with intraoperatively confirmed CSFF and an absent stapedial tendon. INTERVENTIONS: Computed tomography (CT); exploratory tympanotomy with stapedotomy. MAIN OUTCOME MEASURES: Absence of a pyramidal eminence and stapedial tendon aperture identified on preoperative CT that was confirmed intraoperatively. RESULTS: Eight patients with intraoperative confirmation of CSFF and absent stapedial tendon were retrospectively identified. The average preoperative bone conduction and air conduction pure tone averages were 19.6 dB (SD 15.6 dB) and 55.9 dB (SD 23.6 dB), respectively. The average air-bone gap was 36.3 dB (SD 17.9 dB) preoperatively. In the seven patients who underwent preoperative CT, all were consistently identified to have an absent or hypoplastic pyramidal eminence and absent stapedial tendon aperture at the pyramidal eminence. In six cases, the stapedial footplate appeared normal, while in one case the footplate appeared abnormal which correlated with severe facial nerve prolapse observed intraoperatively. All eight cases underwent exploratory tympanotomy and demonstrated intraoperative stapes footplate fixation, absent stapedial tendon and either absent or hypoplastic pyramidal eminence, which correlated with preoperative CT findings. CONCLUSIONS: This study identifies a clinically pragmatic association between an absent pyramidal eminence identified on high-resolution CT and the diagnosis of CSFF. In a condition that otherwise generally lacks distinctive radiological features, the absence of a pyramidal eminence on CT in a patient with nonprogressive, congenital conductive hearing loss may strengthen clinical suspicion for CSFF.

Transtympanic pseudoaneurysm of the internal carotid artery complicating a myringotomy in a four-year old child: Case report and literature review.

We report the first case of a transtympanic iatrogenic internal carotid artery (ICA) pseudoaneurysm diagnosed in a 4-year-old child following a myringotomy. An endovascular treatment with a covered-stent was decided; spontaneous thrombosis was found during the therapeutic arteriography, and the procedure was aborted. Otoscopy and computed tomography (CT) scan monitoring showed a prolonged thrombosis and the disappearance of the pseudoaneurysm 18months after the diagnostic arteriography. Based on literature review, endovascular techniques seem to be preferred to the surgical approach for treatment of intrapetrous ICA pseudoaneurysm, however clinical and CT scan monitoring may also be a valid option.

The relationship between post-traumatic ossicular injuries and conductive hearing loss: A 3D-CT study.

PURPOSE: After a trauma, the conductive ossicular chain may be disrupted by ossicular luxation or fracture. Recent developments in 3D-CT allow a better understanding of ossicular injuries. In this retrospective study, we compared patients with post-traumatic conductive hearing loss (CHL) with those referred without CHL to evaluate the relationship between ossicular injuries and CHL. We also assessed the added value of 3D reconstructions on 2D-CT scan to detect ossicular lesions in patients surgically managed. METHODS: The CT scans were performed using a 40-section spiral CT scanner in 49 patients with post-traumatic CHL (n=29) and without CHL (n=20). Three radiologists performed independent blind evaluations of 2D-CT and 3D reconstructions to detect ossicular chain injury. We used the t-test to explore differences regarding the number of subjects with ossicular injury in the two groups. We also estimated the diagnostic accuracy and the inter-rater agreement of the 3D-CT reconstructions associated to 2D-CT scan. RESULTS: We identified ossicular abnormality in 14 patients out of 29 and in one patient out of 20 in the CHL and non-CHL groups respectively. There was a significant difference regarding the number of subjects with ossicular lesions between the two groups (P≤0.01). The diagnostic sensitivity of 3D-CT reconstructions associated with 2D-CT ranged from 66% to 100% and the inter-reader agreement ranged from 0.85 to 1, depending of the type of lesion. CONCLUSION: The relationship between ossicular lesion and the presence of CHL tightly correlated. 3D-CT reconstructions of the temporal bone are useful to assess patients in a post-traumatic context.

Case 226: Oval Window Atresia.

A 6-year-old girl presented with bilateral hearing loss. Her otologic, birth, and family histories were limited, given that she was adopted, but her parents reported that she had had difficulty hearing and speaking ever since they adopted her at 2 years of age. Her parents denied a history of acute otitis media, otorrhea, otalgia, vertigo, autophony, or tinnitus since her adoption. At 2.5 years of age, a diagnosis of hearing loss was made, and she was given hearing aids. Her parents believed that she had been doing well with both receptive and expressive language since she had received the hearing aids. At examination, she had small bilateral preauricular skin tags and normal pinna. Her external auditory canals were of a normal caliber bilaterally, with no otorrhea or lesions. The tympanic membranes were translucent and mobile at pneumatic otoscopy. There was no evidence of a middle ear lesion, nor was there a Schwartz sign. She had no nystagmus or vertigo at pneumatic otoscopy. Audiometry was performed and revealed moderate to severe conductive hearing loss bilaterally, with a mixed component present at 2000 KHz. She had normal bilateral middle ear pressure at tympanometry. Thin-section computed tomography (CT) of the temporal bone was performed.

Imaging of Conductive Hearing Loss With a Normal Tympanic Membrane.

OBJECTIVE: This article presents an approach to imaging conductive hearing loss in patients with normal tympanic membranes and discusses entities that should be checked as the radiologist evaluates this potentially complicated issue. CONCLUSION: Conductive hearing loss in a patient with a normal tympanic membrane is a complicated condition that requires a careful imaging approach. Imaging should focus on otosclerosis, and possible mimics and potential surgical considerations should be evaluated. The radiologist should examine the ossicular chain and the round window and keep in mind that a defect in the superior semicircular canal can disturb the hydraulic integrity of the labyrinth.

The prevalence of superior semicircular canal dehiscence in conductive and mixed hearing loss in the absence of other pathology using submillimetric temporal bone computed tomography.

OBJECTIVE: The objective of this study was to assess the relationship between superior semicircular canal dehiscence (SSCD) and hearing impairment. METHODS: We retrospectively compared the prevalence of SSCD in the ears classified as conductive hearing loss (CHL), mixed hearing loss (MHL), and normal hearing status using submillimetric temporal bone computed tomography (TBCT) on the basis of coronal and additional reformatted planes dedicated to SSCD. RESULTS: From the patients with CHL (n = 127) and MHL (n = 45), the overall prevalence of SSCD in the ears classified as CHL, MHL, and normal hearing status were 6.6%, 7.2%, and 3.0%, respectively. Furthermore, the odds ratio for SSCD in the absence of any cause of hearing loss (eg, dysfunction of the tympanic membrane or middle ear, TBCT abnormalities, otosclerosis, trauma, surgery) was 5.35 in MHL (4/27; P = 0.037, 95% confidence interval, 1.1-25.81) and 3.31 in CHL (5/61; P = 0.115, 95% confidence interval, 0.75-14.63), compared with normal hearing status. CONCLUSIONS: Bony covering of the SSC should be specifically evaluated in patients with hearing impairment using submillimetric TBCT.

Superior canal dehiscence syndrome: clinical manifestations and radiologic correlations.

The objective of this study is to describe the superior canal dehiscence syndrome (SCDS) and its vestibule-cochlear manifestations, while analyzing dehiscence size, audiogram and vestibular-evoked myogenic potential (VEMP) changes following dehiscence obliteration. We conducted a prospective study in a tertiary referral center. All Patients diagnosed and surgically treated for SCDS were operated through a middle fossa craniotomy (MFC). Clinical and radiological data were collected. The main outcome measures were Air-bone gaps, Pure-tone average (PTA), speech discrimination scores (SDS) and VEMP thresholds and were correlated to dehiscence size. 28 patients were included in this study with a mean dehiscence size of 4.68 mm. Phonophobia and imbalance were the most debilitating cochlear and vestibular symptoms, respectively. At 2 months postoperatively, low-frequency air-bone gaps showed a statistically significant improvement (p < 0.001). SDS and PTA did not show any statistically significant changes 2 months postoperatively (p = 0.282 and p = 0.295, respectively). VEMP threshold differences between operated and contralateral ears were statistically significant preoperatively (p < 0.001) and non-significant 2 months postoperatively (p = 0.173). Dehiscence size only showed a statistically significant correlation with preoperative total cochlear symptoms, while remaining insignificant with all other variables measured. Air-bone gaps, VEMP and computerized tomography remain essential tools in diagnosing and following SCDS. Dehiscence size is an independent factor in the analysis of SCDS, with cochlear symptomatology being associated to dehiscence sizes. Finally, it is shown that overall symptomatology, audiometric results and VEMP thresholds return to normal values post-obliteration, confirming the continuing success of the MFC approach for SCDS obliteration.

Preoperative computed tomography scan may fail to predict perilymphatic gusher.

OBJECTIVES: A stapes gusher is a very rare event in ear surgery, but the consequences for hearing can be dramatic. It can usually be predicted by characteristic radiologic abnormalities. We report 2 cases of gusher without any abnormalities seen on the preoperative computed tomography scans. METHODS: The first case was in a 30-year-old man with a bilateral mixed hearing loss. The gusher occurred after a stapedotomy performed with a microdrill. The oval window was plugged with a vein graft, and a fluoroplastic piston was inserted. The second case was in a 39-year-old woman with a family history of hearing loss who presented with a bilateral mixed hearing loss. The footplate was fractured during the stapedotomy drilling and was covered with a temporalis fascia graft that was fixed with a fluoroplastic piston. RESULTS: The first patient had no cerebrospinal fluid leakage and no vertigo or tinnitus. He did have a sensorineural hearing loss. The second patient had dizziness and tinnitus. Postoperative magnetic resonance imaging scans were performed, but again no features were identified that might have predicted these cases. CONCLUSIONS: Surgeons should be reminded that a preoperative computed tomography scan may fail to detect the risk of a perilymphatic gusher.

Crural attachment to promontory case report: implications for stapes development.

Understanding of the embryologic origin of the stapes remains controversial. Theories diverge upon whether the entirety of the stapes arises from a single source versus the footplate and suprastructure arising from distinct sources. A 12-year-old boy with left-sided conductive hearing loss had computed tomography of the temporal bone, revealing an inferiorly displaced left stapes, and a nonspecific density in the left Prussak's space. Exploratory tympanotomy revealed the crura of the stapes to be attached to the promontory. The stapes footplate was located in the oval window and was mobile.

Audiological and surgical evidence for the presence of a third window effect for the conductive hearing loss in DFNX2 deafness irrespective of types of mutations.

The objective of this study was to clarify the cause of the air-bone gap in incomplete partition (IP) type III cases according to the POU3F4 gene (DFNX2) mutation type. A retrospective analysis of patient medical records was done in a tertiary referral medical center. Five IP type III patients proved to be carrying a mutation in or affecting POU3F4. The hearing and the middle ear status at either exploratory tympanotomy or cochlear implantation from these DFNX2 cases was reviewed. Four of five unrelated IP type III patients harbored a point mutation of POU3F4 and the fifth patient carried a large genomic deletion upstream to POU3F4. Two of the four DFNX2 patients carrying a point mutation had moderate to severe mixed hearing loss with a substantial amount of air-bone gap. These patients underwent exploratory tympanotomy to identify the cause of their hearing loss. The other three patients, including one carrying a large deletion, had profound hearing loss at presentation and received a cochlear implant. In the exploratory tympanotomy group with a substantial amount of air-bone gap and a point mutation (n = 2), one patient had a perfect ossicular chain with normal mobility, a positive ipsilateral stapedial reflex, and a positive round window reflex. In the cochlear implantation group (n = 3), we found a stapes with normal mobility and a positive round window reflex in one patient who harbored a large genomic deletion upstream to POU3F4. We concluded that the probable presence of the third window effect is not limited to the particular type of POU3F4 mutation.

Does microtia predict severity of temporal bone CT abnormalities in children with persistent conductive hearing loss?

PURPOSE: This study aimed to determine the spectrum of temporal bone computed tomography (CT) abnormalities in children with conductive hearing loss (CHL) with and without microtia. PATIENTS AND METHODS: From 1993 to 2008, a total of 3396 pediatric records including CHL were reviewed at our institution and revealed 180 cases of persistent CHL, 46 of whom had diagnostic temporal bone CT examinations. All of these examinations were systematically reviewed by two pediatric neuroradiologists, working in consensus, who had 5 and 18 years, respectively, of dedicated pediatric neuroradiology experience. RESULTS: Of the 46 children, 16 were boys and 30 were girls (age: 0.2-16 years; mean: 5 years). Also, 21 (46%) children had microtia and 25 (54%) children did not, as determined by clinical evaluation. External auditory canal atresia/stenosis (EAC-A/S) was the most common anomaly in both microtia and non-microtia groups. Two or more anomalies were observed in 18/21 children with microtia. The frequency of EAC-A/S was greater in children with microtia versus those without it (86% versus 32%, respectively; P = 0.0003). Syndromic diagnoses were also significantly more frequently made in children with microtia versus those without microtia (76% versus 20%, respectively; P = 0.0001). Temporal bone CT scans were normal in 10 children (22%) with persistent CHL. CONCLUSION: Microtia is an important finding in children with CHL. EAC and middle ear/ossicle anomalies were significantly more frequently seen in children with microtia, and multiple anomalies and bilateral microtia were more common in children with syndromic associations. These findings highlight the importance of understanding the embryological development of the temporal bone. The presence of one anomaly should raise suspicion of the possibility of other anomalies, especially in the setting of microtia. Bilateral microtia and multiple anomalies should also raise suspicion of genetic syndromes.

Conductive Hearing Loss in Children.

A variety of congenital and acquired disorders result in pediatric conductive hearing loss. Malformations of the external auditory canal are invariably associated with malformations of the middle ear space and ossicles. Isolated ossicular malformations are uncommon. Syndromes associated with external and middle ear malformations are frequently associated with abnormal development of first and second pharyngeal arch derivatives. Chronic inflammatory disorders include cholesteatoma, cholesterol granuloma, and tympanosclerosis.

Inner ear anomalies seen on CT images in people with Down syndrome.

BACKGROUND: Although dysplasia of inner ear structures in Down syndrome has been reported in several histopathological studies, the imaging findings have not been widely studied. OBJECTIVE: To evaluate the prevalence and clinical significance of inner ear anomalies detected on CT images in patients with Down syndrome. MATERIALS AND METHODS: The temporal bone CT images of patients with Down syndrome were assessed for inner ear anomalies; clinical notes and audiograms were reviewed for hearing loss. Logistic regression models were employed to identify which CT findings were associated with sensorineural hearing loss (SNHL). RESULTS: Inner ear anomalies were observed in 74.5% (38/51) of patients. Malformed bone islands of lateral semicircular canal (LSCC), narrow internal auditory canals (IACs), cochlear nerve canal stenoses, semicircular canal dehiscence (SCCD), and enlarged vestibular aqueducts were detected in 52.5% (53/101), 24.5% (25/102), 21.4% (21/98), 8.8% (9/102) and 2% (2/101) of patients' ears, respectively. IAC stenosis had the highest odds ratio (OR = 5.37, 95% CI: 1.0-28.9, P = 0.05) for SNHL. CONCLUSION: Inner ear anomalies occurred in 74.5% of our population, with malformed (<3 mm) bone island of LSCC being the most common (52.5%) anomaly. Narrow IAC was seen in 24.5% of patients with Down syndrome and in 57.1% of ears with SNHL. High-resolution CT is a valuable for assessing the cause of hearing loss in people with Down syndrome.

Ear and further anatomic anomalies in children undergoing stapedotomy.

PURPOSE: Aim of this study was to examine any stapes fixation-related clinical, intraoperative and high-resolution computed tomography (HRCT) findings in children undergoing stapedotomy. PROCEDURES: Detailed diagnostic assessment was performed in 12 children (18 ears) who underwent stapedotomy within the period 2005-2008. RESULTS: Ear malformations such as malleus-incus anomalies, malleus-epitympanum fixation and absence of the stapedius tendon, small external auditory canal and auricle malformations were clinically and/or intraoperatively diagnosed. HRCT identified a petrous high jugular bulb and malformed inner auditory canal in 4 ears; however, it failed to recognize ossicular fixation. Systemic anomalies, namely kyphoscoliosis, esophageal atresia and finger malformations were identified in 2 children. Summing up, additional anatomic anomalies were found in 8 patients (66.7%). CONCLUSIONS: Ear malformations may coexist in children with stapes fixation. Ossicular fixation is not easily recognized with HRCT, and therefore, ossicular mobility should always be tested intraoperatively. In a few cases, pediatric stapes fixation can coexist with systemic dysplasias; in such patients, additional referral to geneticists is recommended.

Isolated Incudostapedial Cholesteatomas: Unique Radiologic and Surgical Features.

OBJECTIVES: Congenital cholesteatomas originate from epithelial tissue present within the middle ear in patients with an intact tympanic membrane, no history of otologic surgery, otorrhea, or tympanic membrane perforation. They are diagnosed by a pearl-like lesion on otoscopy and computed tomography (CT) scan showing an expansile soft-tissue mass. We describe a series of patients with no prior otologic history presenting with progressive unilateral conductive hearing loss and normal otoscopy. The CT scans showed ossicular erosion without obvious soft-tissue mass. Surgery confirmed incudostapedial erosion found to be cholesteatoma. In this study, we characterize the clinical course of patients diagnosed with isolated incudostapedial cholesteatoma (IIC) and review possible pathologic mechanisms. METHODS: Retrospective review of IIC cases treated by the Department of Pediatric Otolaryngology, Rady Children's Hospital, San Diego, 2014 to 2020. Data included patient demographics, clinical features, imaging, surgical findings, and audiologic data. RESULTS: Five patients were diagnosed with IIC (3 [60%] female; mean age at presentation 10.7 years [range 5.5-16.0]). All patients presented with postlingual unilateral conductive hearing loss and normal otoscopy without any past otologic history; delay in diagnosis ranged from 4 months to several years. The CT scans showed ossicular chain erosion with an absent long process of the incus and/or stapes superstructure. All patients underwent middle ear exploration, revealing a thin layer of cholesteatoma in the incudostapedial region, confirmed by histopathology. Mean preoperative speech reception threshold was 55 dB and improved to a mean of 31 dB in the 4 patients who underwent ossicular chain reconstruction. CONCLUSION: Isolated incudostapedial cholesteatoma should be included as a possible etiology in pediatric patients with insidious onset of unilateral conductive hearing loss with normal otoscopy, unremarkable otologic history, and a CT scan showing ossicular abnormality/disruption without notable middle ear mass. These patients should be counseled preoperatively regarding the possibility of cholesteatoma and should undergo middle ear exploration with possible ossiculoplasty.

Ear-pick injury as a traumatic ossicular damage in Japan.

This retrospective study examined the etiology and treatment results for traumatic, conductive hearing loss in 22 patients who underwent surgery between 1998 and 2008 at Osaka Red Cross Hospital. All patients underwent computed tomography (CT) of the temporal bone preoperatively. The ear surgery comprised closure of the ruptured tympanic membranes and restoration of the sound-transmitting function of the ossicular chain. Their mean age was 30.3 years, and the average delay from injury until treatment was 6.0 years. Of the injuries due to foreign-body insertion, the most common cause was ear-pick injury. Incudostapedial disarticulation was the most common finding, which was diagnosed preoperatively using CT in seven cases and identified at surgery in 15 cases. Closure of the air-bone gap to within 10 and 20 dB was observed in 50.0 and 68.2% of the patients, respectively. The hearing threshold improved by 10 dB or more in 16 (72.7%) patients. If no improvement in hearing loss follows the absorption of hemotympanium or closure of an eardrum perforation, dislocation of the ossicular bones should be suspected. Ossicular reconstruction following trauma produces more stable and better hearing results, even after delayed treatment.

What the surgeon cannot see and needs to see before middle ear surgery.

OBJECTIVE: To define the utility of imaging prior to middle ear surgery. METHODS: Different middle ear pathologies are subsequently described: conductive hearing loss with a normal tympanic membrane, labyrinthine fistulae, tegmen anomalies, opacified tympanic membrane, cholesteatoma, temporal bone fractures and glomus tumors. We discuss the indications and benefits of imaging in each of these pathologies. RESULTS: Preoperative imaging shows interesting features and can be very helpful in the differential diagnosis and in surgical decision-making and planning. However, it is not mandatory and should not be systematic in every middle ear surgery. CONCLUSION: The modern radiological era provides the otological surgeon with numerous imaging technologies. Yet, these techniques should by no means replace the surgeon's anatomical knowledge, clinical sense and surgical skills.

[High-resolution CT of temporal bone trauma: review of 38 cases]. / L'apport du scanner dans les traumatismes du rocher: à propos de 38 cas.

PURPOSE: Temporal bone trauma is frequent but difficult to assess due to the diversity of clinical presentations and complex anatomy. We have sought to assess the different types of fractures and complications on high-resolution CT. Materials and methods. Descriptive retrospective study over a 24 month period performed in the ENT radiology section of the Mohammed VI university medical center in Marrakech. A total of 38 cases of temporal bone trauma were reviewed. All patients underwent ENT evaluation and high-resolution CT of the temporal bone using 1mm axial and coronal sections. RESULTS: Mean patient age was 33 years (range: 14-55 years) with male predominance (sex ratio: 36/2). Clinical symptoms were mainly otorrhagia and conductive hearing loss. Oblique extra-labyrinthine fractures were most frequent. Two cases of pneumolabyrinth were noted. Management was conservative in most cases with deafness in 3 cases. CONCLUSION: High-resolution CT of the temporal bone provides accurate depiction of lesions explaining the clinical symptoms and helps guide management. MRI is complimentary to further assess the labyrinth and VII-VIII nerve complex.

High Resolution Computed Tomography (HRCT) Imaging Findings of Oval Window Atresia with Surgical Correlation.

INTRODUCTION: Isolated oval window atresia (OWA) is a rare cause of congenital conductive middle ear deafness and may be overlooked owing to the normal appearance of the external ear. This anomaly has been previously described, although the published numbers with both imaging and surgical findings are few. Our aim is to correlate the imaging features of OWA with intraoperative findings. MATERIALS AND METHODS: This is a single-centre retrospective evaluation of patients who were diagnosed with OWA and who received surgery from January 1999 to July 2006. No new case was diagnosed after 2006 to the time of preparation of this manuscript. High resolution computed tomography (HRCT) imaging of the temporal bones of the patients were retrospectively evaluated by 2 head and neck radiologists. Images were evaluated for the absence of the oval window, ossicular chain abnormalities, position of the facial nerve canal, and other malformations. Imaging findings were then correlated with surgical findings. RESULTS: A total of 9 ears in 7 patients (two of whom with bilateral lesions) had surgery for OWA. All patients had concomitant findings of absent stapes footplate with normal, deformed or absent stapes superstructure and an inferiorly displaced facial nerve canal. HRCT was sensitive in identifying OWA and associated ossicular chain and facial nerve abnormalities, which were documented surgically. CONCLUSION: OWA is a rare entity that can be diagnosed with certainty on HRCT, best visualised on coronal plane. Imaging findings of associated middle ear abnormalities, position of the facial nerve canal, which is invariably mal-positioned, and associated deformity of the incus are important for presurgical planning and consent.

Implantation of bone-anchored hearing device using a three-dimensional template in a child.

BACKGROUND: Implantation of bone-anchored hearing devices is performed to improve hearing in patients with chronic suppurative otitis media who cannot wear a conventional hearing aid. The surgical procedure can be safely performed in children aged over five years. CASE REPORT: A 15-year-old patient with bilateral chronic suppurative otitis media and conductive hearing loss underwent the procedure to implant a bone-anchored hearing device but was found to have skull thickness of less than 2.5 mm and the procedure was abandoned. A computed tomography scan of the skull was undertaken and a three-dimensional template was reconstructed to identify appropriate thickness of the skull to implant the abutment during a second procedure. CONCLUSION: Bone-anchored hearing devices can be implanted by prior imaging and using a template to identify the area of appropriate skull thickness to implant the abutment safely.