[Imaging evaluation of cerebrospinal fluid otorrhea associated with inner ear malformation in children].

Objective: To explore the imaging evaluation of cerebrospinal fluid (CSF) otorrhea associated with inner ear malformation (IEM) in children. Methods: The clinical data of 28 children with CSF otorrhea associated with IEM confirmed by surgical exploration in Beijing Children's Hospital, from Nov, 2016 to Jan, 2021, were analyzed retrospectively,including 16 boys and 12 girls, aged from 8-month to 15-year and 8-month old, with a median age of 4-year old. The shapes of stapes were observed during the exploration surgery, and the imaging features of temporal bone high resolution CT(HRCT) and inner ear MRI pre- and post-operation were analyzed. Results: In 28 children with CSF otorrhea, 89.3%(25/28) had stapes footplates defect during exploration. Preoperative CT showed indirect signs such as IEM, tympanic membrane bulging, soft tissue in the tympanum and mastoid cavity. IEM included four kinds: incomplete partition type I (IP-Ⅰ), common cavity (CC), incomplete partition type Ⅱ (IP-Ⅱ), and cochlear aplasia (CA); 100%(28/28) presented with vestibule dilation; 85.7%(24/28) with a defect in the lamina cribrosa of the internal auditory canal. The direct diagnostic sign of CSF otorrrhea could be seen in 73.9%(17/23) pre-operative MRI: two T2-weighted hyperintense signals between vestibule and middle ear cavity were connected by slightly lower or mixed intense T2-weighted signals, and obvious in the coronal-plane; 100%(23/23) hyperintense T2-weighted signals in the tympanum connected with those in the Eustachian tube.In post-operative CT, the soft tissues in the tympanum and mastoid cavity decreased or disappeared as early as one week. In post-operative MRI, the hyperintense T2-weighted signals of tympanum and mastoid decreased or disappeared in 3 days to 1 month,soft tissues tamponade with moderate intense T2-weighted signal were seen in the vestibule in 1-4 months. Conclusions: IP-Ⅰ, CC, IP-Ⅱ and CA with dilated vestibule can lead to CSF otorrhea. Combined with special medical history, T2-weighted signal of inner ear MRI can provide diagnostic basie for most children with IEM and CSF otorrhea.HRCT and MRI of inner ear can also be used to evaluate the effect of surgery.

Comprehensive review of the mastoid foramen.

Anatomical variations of the mastoid foramen have been observed to vary in a number of qualities including size, number, and location. These variants have the potential to become problematic during surgical approaches to the posterior cranial fossa and mastoid part of the temporal bone, and should thus be appreciated by the surgeon. Herein, we discuss the mastoid foramen in detail including issues with such foramina that should be known to the neurosurgeon.

A Novel Classification: Anomalous Routes of the Facial Nerve in Relation to Inner Ear Malformations.

OBJECTIVES/HYPOTHESIS: The objective of this study was to classify anomalous facial nerve (FN) routes and to determine their association with inner ear malformations (IEMs). STUDY DESIGN: Retrospective cross sectional study. METHODS: The computed tomography images of 519 patients (796 ears) with IEMs were retrospectively evaluated, and the abnormal routes of the FN were classified as: Meatal segment: type 1, normal internal auditory canal (IAC); type 2, narrow IAC; type 3, facial canal (FC) only; type 4: separate FC/duplicated IAC. Labyrinthine segment (LS): type 1, normal; type 2a/b/c, mild/moderate/severe anterior displacement; type 3, superior displacement; type 4: straight LS. Tympanic segment (TS): type 1, normal; type 2, superiorly displaced TS; type 3, TS at the oval window; type 4: TS inferior to the oval window; type 5: unclassified. Mastoid segment: type 1, normal facial recess (FR)/normal mastoid segment; type 2: narrow FR; type 3, unclassified. RESULTS: In meatal segment classification, a narrow IAC was common in ears with cochlear hypoplasia (CH) (76.1%), and only FC was common in ears with severe IEMs (62.7%) such as Michel deformity, common cavity, and cochlear aplasia. Incomplete partition-III has its unique superiorly displaced LS (100%). CH-IV also has its unique mild anterosuperior displacement. Ears with a superiorly displaced TS usually (93.1%) had aplastic or hypoplastic semicircular canals. The FR is likely to be narrow in CH and severe IEMs. CONCLUSIONS: The FN route is affected in IEMs, which must be kept in mind when operating on ears with IEMs. Especially in CH cases, all segments of the FN can be abnormal. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:E696-E703, 2020.

Variation of the sternocleidomastoid muscle: a case report of three heads and an accessory head.

PURPOSE: Anatomical variations of the sternocleidomastoid muscle (SCM) have been observed to occupy multiple origins and insertion points and have supernumerary heads, sometimes varying in thickness. METHODS: An 85-year-old Korean male cadaver was dissected and a SMC with unilateral supernumerary heads was identified. RESULTS: Three heads and an accessory belly of the sternal head were recorded on the left side. There were two clavicular heads, one lateral and one medial. The sternal head originated inferior to the manubrium of the sternum. A small belly arose from the sternal head and merged to the lateral clavicular head on the C5 vertebrae level. These bellies inserted into the mastoid process of the temporal bone. CONCLUSIONS: These variants may be problematic during surgical approaches to the upper neck and occipital, and should thus be appreciated by the clinician.

Transmastoid access in branchio-oto-renal syndrome: A reappraisal of computed tomography imaging.

OBJECTIVE: To evaluate for temporal bone abnormalities that might affect transmastoid surgery such as cochlear implantation in cases of branchio-oto-renal syndrome (BOR). STUDY DESIGN: Retrospective review. METHODS: Qualitative assessment of temporal bone computed tomography imaging was performed by a neuroradiologist for 30 individuals with BOR (60 ears) and 20 controls with normal hearing (20 ears). Transmastoid access was assessed categorically across 4 features: tip development, cortex pneumatization, tegmen height, and facial recess pneumatization. The appearance of 4 standard landmarks (Koerner's septum, antrum, prominence of the horizontal semicircular canal, incudal short process) was also dichotomized as normal or abnormal. Data were compared using Fisher's exact testing. RESULTS: Mastoid height differed between the groups with tip underdevelopment noted in 72% of BOR ears vs. 40% of controls (p = 0.02), and a low tegmen was seen in 68% of BOR ears and 25% of controls (p < 0.01). Significant differences in pneumatization were also found for the mastoid cortex (28% non-pneumatized in BOR vs. 5% in controls; p = 0.03) and the facial recess (27% non-pneumatized in BOR vs. 0% in controls; p = 0.01). Standard landmarks were easily identified in all of the control mastoids. In the BOR group, Koerner's septum was abnormally located or absent in 45%, and the antrum was severely hypoplastic or absent in 50%. Similarly, the prominence of the horizontal semicircular canal and the short process of the incus were dysplastic in 73% (44/60) and 62% (37/60), respectively. CONCLUSIONS: Mastoid abnormalities are common in BOR syndrome. Restricted transmastoid access and abnormal or absent mastoid landmarks should be anticipated in those patients with BOR who become cochlear implant candidates. LEVEL OF EVIDENCE: 4.

CT findings of the temporal bone in CHARGE syndrome: aspects of importance in cochlear implant surgery.

To provide an overview of anomalies of the temporal bone in CHARGE syndrome relevant to cochlear implantation (CI), anatomical structures of the temporal bone and the respective genotypes were analysed. In this retrospective study, 42 CTs of the temporal bone of 42 patients with CHARGE syndrome were reviewed in consensus by two head-and-neck radiologists and two otological surgeons. Anatomical structures of the temporal bone were evaluated and correlated with genetic data. Abnormalities that might affect CI surgery were seen, such as a vascular structure, a petrosquamosal sinus (13 %), an underdeveloped mastoid (8 %) and an aberrant course of the facial nerve crossing the round window (9 %) and/or the promontory (18 %). The appearance of the inner ear varied widely: in 77 % of patients all semicircular canals were absent and the cochlea varied from normal to hypoplastic. A stenotic cochlear aperture was observed in 37 %. The middle ear was often affected with a stenotic round (14 %) or oval window (71 %). More anomalies were observed in patients with truncating mutations than with non-truncating mutations. Temporal bone findings in CHARGE syndrome vary widely. Vascular variants, aberrant route of the facial nerve, an underdeveloped mastoid, aplasia of the semicircular canals, and stenotic round window may complicate cochlear implantation.

Lambdoid Synostosis Versus Positional Posterior Plagiocephaly, a Comparison of Skull Base and Shape of Calvarium Using Computed Tomography Imaging.

The differential diagnostics between the common positional posterior plagiocephaly and relatively rare lambdoid synostosis is important due to the differences in their treatment plan and clinical management. However, the clinical criteria for the diagnosis of lambdoid synostosis are not clear since there is a considerable overlap in the features of positional posterior plagiocephaly and unilateral lambdoid synostosis. To systematically evaluate the clinical findings in these 2 patient groups, we quantitatively compared the characteristics of endocranial skull base and ectocranial calvarium in 3D computed tomography, in 9 children (mean age 2.9 years) with unilateral lambdoid synostosis and 9 children with positional posterior plagiocephaly. The groups were sex and age matched. Our results show that the skull bases in the lambdoid synostosis are posteriorly shorter and more twisted than in positional posterior plagiocephaly. Anterior twisting was mild in both skull types. Our study confirmed earlier suggested diagnostic feature: prominent ipsilateral mastoidal bossing downward and laterally in all lambdoid skulls. In positional posterior plagiocephaly the bossing was typically not detected. Interestingly, there was a great variation in the position of the ipsilateral ear and external auditory meatus in both patient groups. Thus, neither antero-posterior nor vertical position of ear is a reliable differential diagnostic feature between lambdoid synostosis or positional posterior plagiocephaly.

The extent of inferior displacement of the mastoid tegmen is related to the severity of congenital aural atresia.

CONCLUSION: The extent of inferior displacement of the mastoid tegmen is related to the severity of congenital aural atresia (CAA). OBJECTIVE: To analyze anatomic variations observed on high-resolution temporal bone computed tomography (TBCT) in patients with CAA, the extent of inferior displacement of the mastoid tegmen and the size of the incudo-stapedial (IS) joint angle were compared with surgical parameters for atresiaplasty, such as Jahrsdoerfer score and hearing acuity. METHODS: Sixty-one patients with unilateral CAA underwent high-resolution TBCT and hearing tests. We evaluated TBCTs in terms of Jahrsdoerfer criteria and analyzed the relationships among the inferior displacement of the mastoid tegmen, size of the IS joint angle, hearing acuity, and the Jahrsdoerfer score on the atretic side. RESULTS: IS joint angle on the atretic side was found to be 120.6 ± 11°, which was significantly greater than the corresponding value of 103.4 ± 5.4° on the normal side. Cholesteatoma occurred significantly more often in CAA patients with narrow external auditory canals (EACs) (9/27; 33.3%), compared with CAA patients with no EAC opening (2/34; 5.9%). There was a significant relation between the extent of inferior displacement of the mastoid tegmen (28.9% in the atretic side and 17.3% in the normal side) and the Jahrsdoerfer score (p < 0.0001).

Congenital aplasia of the external ear canal and total dehiscence of mastoid-tympanic segment of the facial nerve as a cause of recurrent facial paralysis.

Facial nerve (FN) macrodehiscence, in contrast to microdehiscence, generally occurs as a result of chronic otitis media and is rarely seen congenitally. A patient with normal hearing who had no history of ear disease or ear operation came to the clinic with recurrent facial paralysis (FP) and frequent blockage of the external ear canal with epithelial debris complaints. In the explorative ear surgery, we observed that the posterior wall of the external ear canal or tympanic ring was absent, a large external ear cavity was covered with a thin skin, the FN was under the skin, and the tympanic-mastoid segment of the FN and chorda tympani extending to the stylomastoid foramen was completely open. FP episodes were associated with the unprotected FN.To prevent an attack of FP, and to self-clean the external ears, mastoid obliteration surgery was performed, and the mastoid segment of the FN was covered with a conchal cartilage graft. This case was diagnosed as congenital aplasia of the external ear canal due to the tympanic bone aplasia-mastoid bone hypoplasia, and the dehiscence of the mastoid-tympanic segment of the FN, and as a cause of recurrent FP, has never to date been identified.

Goldenhar syndrome.

Goldenhar syndrome is a syndrome of complex structures developing from first and second branchial arches during blastogenesis. The etiology of this rare disease is not fully understood, as it has shown itself variable genetically and of unclear causes. The disorder is characterized by a wide spectrum of symptoms and physical features that may vary greatly in range and severity from case to case. Here we present a unique case of Goldenhar syndrome with absence of left condyle, hypoplasia of the zygomatic bone, no pneumatization of the mastoid process, underdeveloped mandible, bifid tongue and the skin tags in the preauricular area.

Primary mastoid cyst.

OBJECTIVE: To report an unusual case of a primary mastoid cyst (congenital or developmental) in a patient without otological symptoms. METHOD: Case report and review of the English language literature. RESULTS: Primary mastoid cyst is a newly reported and very rare pathological entity. Mastoid cysts usually occur secondary to chronic infection, inflammation or trauma. Review of the medical literature highlights the rarity of this condition. CONCLUSION: This report describes the experience gained by the diagnosis and management of this patient. It emphasises the importance of clinical vigilance so that proper treatment may be instituted in a timely manner.

The developmental characteristics of mastoid pneumatisation in cleft palate children: the genetic influence.

Physiologic and developmental role of mastoid pneumatisation in children with otitis media with effusion (OME) is still controversial. For measuring mastoid pneumatisation and examine developmental characteristics, we used children with orofacial malformation of high risk for long term negative pressure in the middle ear and are expected to have lower rate of size and growth of pneumatisation. Mastoid were measured on Schuller's mastoid X-ray pictures planimetrically in study group of 146 children with bilateral (BCLP), unilateral (UCLP) and isolated (ICP) cleft palate, and control group of non-cleft 52 children, both groups with confirmed otitis media with effusion and no previous otological surgery. The lowest pneumatisation found in BCLE, BCLP and UCLP showed no growth of mastoid with age and lower mastoid size than OME controls. ICP is the only cleft type with growth of mastoid with aging. OME patients has the highest size of mastoid and growth rate with aging.

Discontinuity of the incudo-stapedial joint within a fully aerated middle ear and mastoid on computed tomography: A clinico-radiological study of its aetiology and clinical consequence.

AIM: To investigate the aetiology and clinical consequences of incudo-stapedial (IS) discontinuity when it is demonstrated on computed tomography (CT) within a fully aerated middle ear and mastoid. METHODS AND MATERIALS: Patients with CT evidence of IS discontinuity within a fully aerated middle ear and mastoid were prospectively identified. Clinical history, otoscopic findings, audiometry, and CT data were evaluated. Predefined criteria were used to determine the likely aetiology of IS discontinuity, whether it was diagnosed prior to the CT study, and the clinical consequences in terms of degree of conductive hearing loss and requirement for surgical correction. The range of CT appearances was evaluated. RESULTS: The IS discontinuity in 34/36 ears was felt to be due to incus erosion secondary to chronic otitis, on the basis of clinical history and otoscopic findings. The IS discontinuity was rarely evident prior to CT with long-process deficiency being identified in only 5/36 cases. The mean air bone gap was only 22.5 dB. The ossicular defect was surgically addressed in only four cases. The incus deficiency was confined to the lower-third on CT in 19/36 cases. CONCLUSION: When IS discontinuity is demonstrated within a fully aerated middle ear and mastoid, the most likely aetiology is of acquired incus erosion due to chronic otitis media. The IS discontinuity on CT is usually not evident otoscopically. It usually results in only mild conductive hearing loss and the ossicular discontinuity was rarely surgically addressed in the present series.

3p deletion syndrome: implications for cochlear implantation.

OBJECTIVE AND IMPORTANCE: To describe the role of cochlear implantation in patients with 3p deletion syndrome. Additionally, this paper will review non-mastoid approaches to the cochlea potentially used in cases of mastoid abnormalities. CLINICAL PRESENTATION: A 23-month-old child with 3p deletion syndrome (3pDS) presented with suspected hearing loss and characteristic craniofacial abnormalities. Bilateral sensorineural hearing loss (SNHL) was confirmed. Temporal bone computed tomography showed poorly developed mastoids bilaterally. INTERVENTION: Cochlear implantation was successfully undertaken using a retro-auricular transmeatal approach. CONCLUSION: SNHL is commonly encountered in patients with 3pDS and can be improved by cochlear implantation. In this patient population, particular attention must be given to pre-operative imaging and possible altered mastoid anatomy may exist. In doing so, intra-operative complications can be avoided.

Focal defect of mastoid bone shell in the region of the transverse-sigmoid junction: a new cause of pulsatile tinnitus.

OBJECTIVE: Pulsatile tinnitus usually originates from vascular structures, causing an arterial or venous bruit. We report a new cause of pulsatile tinnitus: a focal defect of the mastoid bone shell in the region of the transverse-sigmoid junction, with a normal transverse-sigmoid sinus. PARTICIPANTS AND INTERVENTION: Three patients complained of unilateral, pulsatile tinnitus present for many years. They were identified as having a focal defect of the mastoid bone shell in the region of the transverse-sigmoid junction. The patients underwent transmastoid reconstruction of the mastoid bone shell overlying the transverse-sigmoid sinus. RESULTS: All three patients reported immediate resolution of their symptoms, and were asymptomatic at final follow up. CONCLUSION: A focal defect of the mastoid bone shell overlying the transverse-sigmoid sinus, with no abnormality of the sinus itself, may be a new cause of pulsatile tinnitus. Surgical reconstruction of the mastoid bone shell overlying the transverse-sigmoid sinus can provide lasting symptom relief for patients with pulsatile tinnitus and computed tomographic evidence of the defect.

Chronic, otogenic, epidural pneumatocoele with delayed mass effect: case report.

INTRODUCTION: Mastoid hyperpneumatisation predisposes to intracranial pneumatocoele development, due to the risk of rupture of the thin, bony walls. Intracranial pneumatocoele may be precipitated by even minor head trauma or an abrupt change in middle-ear pressure, with the potential risk of infectious or compressive intracranial complications. CASE REPORT: A 19-year-old man with mastoid hyperpneumatisation developed a chronic intracranial-epidural pneumatocoele of traumatic origin in the right parieto-occipital area, in contiguity with the posterior mastoid cells. Eighteen months later, after a common cold, the patient developed signs of intracranial hypertension, due to the pneumatocoele spreading to the right epidural anterior fossa. A large right mastoidectomy extended to the retrosigmoid cells was performed, and a watertight seal applied over a large retrosigmoid cell using bovine pericardium and a mixture of bone powder and fibrin glue. RESULTS: The patient was discharged on post-operative day three with no symptoms. Ten days after surgery, computed tomography monitoring showed complete reabsorption of the pneumatocoele. CONCLUSION: In cases of chronic, otogenic, epidural pneumatocoele, the possibility of the sudden onset of serious complications suggests the need for early repair of the communication between the temporal bone and the intracranial compartments. Closure of the fistula using autogenic and/or allogenic materials is usually adequate to resolve the pneumatocoele.

Comparison of computed tomographic imaging measurements with clinical findings in children with unilateral lambdoid synostosis.

BACKGROUND: In this study, the authors retrospectively reviewed 12 children with unilateral lambdoid synostosis to compare and contrast clinical findings and associated computed tomographic scan measurements for consistency. METHODS: Multidisciplinary chart records were reviewed for descriptive findings of unilateral lambdoid synostosis. The preoperative computed tomographic scans underwent quantitative analysis of craniofacial morphology. The described clinical findings were compared with the associated computed tomographic measurements for consistency. RESULTS: Clinical findings of unilateral lambdoid synostosis that were consistent with computed tomographic measurements included mastoid cant and bulge, and inferior ear position. Ipsilateral posterior ear position was clinically noted in half of the cases but was anterior in all cases on computed tomographic analysis. Trapezoid head shape was recorded in 11 of the 12 children but present in only eight computed tomographic scans. Median skull base deviation was 9 degrees for the posterior cranial fossa, 10 degrees for the middle cranial fossa, 6 degrees for mastoid cant, and 1 degree for facial twist. There was no correlation between degree of deformity and extent of suture fusion. CONCLUSIONS: Mastoid bossing, skull base cant, and inferior ear deviation ipsilateral to the fused sutures were clinical findings that were consistent with computed tomographic measurements in these patients. Trapezoid head shape and posterior ear deviation were not. Radiographic suture fusion was observed in all cases; however, the extent of fusion did not correlate with the degree of deformity.

Asymmetric agenesis of the mastoid antrum in a neonate.

The authors report a case of asymmetric development of the mastoid antrum in a neonate. The lack of mastoid development most likely represents a congenital maldevelopment. Agenesis of the mastoid antrum has surgical implications, as failure to recognize its absence can result in disorientation during surgery, with potential to injure adjacent structures.

Active middle ear implants in patients undergoing subtotal petrosectomy: new application for the Vibrant Soundbridge device and its implication for lateral cranium base surgery.

OBJECTIVE: The functional outcome of ossiculoplasties in chronic ear and lateral cranium base surgery depends on the presence of a ventilated middle ear space and is guided by the existence or absence of ossicular remnants. In patients with poorly ventilated middle ears, after multiple previous operations, missing stapes suprastructure, or after partial temporal bone resection for tumor removal, restoration of conductive hearing is not possible. The direct placement of a vibrating floating mass transducer (FMT) onto the round window membrane with obliteration of the surgical cavity is a new option. PATIENTS AND INTERVENTION: Starting in January 2006, five patients underwent a subtotal petrosectomy to control their chronically discharging ear, to remove residual cholesteatoma, or to revise previous incompletely exenterated cavities. Four patients underwent a simultaneous placement of a Vibrant Soundbridge (VSB) onto the round window membrane; one patient had a staged reconstruction after initial Bone-Anchored Hearing Aid rehabilitation. In all operations, the external ear canal and the eustachian tube were closed, and the cavity was obliterated using abdominal fat. MAIN OUTCOME MEASURES: Preoperative and postoperative pure tone audiograms were analyzed in respect to deterioration of inner ear function, aided and unaided (hearing aid, VSB, and Bone-Anchored Hearing Aid) speech audiograms were compared to verify improvements in communication skills, functional gains were calculated at comfortable level settings, and postoperative computed tomographic scans were used to exclude recurrent disease and to confirm the position of the FMT onto the round window membrane. Patient's satisfaction was measured using a standardized questionnaire. RESULTS: All patients were very satisfied daily users of their middle ear implant and had complete eradication of their middle ear pathology. Bone conduction worsened at 2 kHz, with preservation of inner ear function in the other frequencies. Whereas none of the patients had any unaided speech discrimination before the surgery at conversational levels, all patients obtained 95 to 100% correct monosyllabic scores at 70 to 80 dB using the VSB. The functional gain was highest at higher frequencies. CONCLUSION: Patients with combined hearing loss undergoing subtotal petrosectomy with complete fat obliteration of the middle ear and mastoid area can be safely rehabilitated, placing the FMT of a VSB onto the round window membrane, either at the time of primary surgery, or as a staged secondary procedure.