Otolaryngologic Features of Craniometaphyseal Dysplasia

1987 ◽  
Vol 96 (6) ◽  
pp. 548-553 ◽  
Author(s):  
G. Robert Kletzker ◽  
Peter G. Smith ◽  
Thomas E. Killeen

Craniometaphyseal dysplasia, one of several rare familial systemic bone disorders of the osteopetrosis bone-disease group, is characterized by abnormal bony thickening of the skull and multiple cranial neuropathies. Hearing loss, commonly of a mixed type, is the usual symptom presented to the otolaryngologist—head and neck surgeon; however, recurring facial paralysis, blindness, and atypical fades may also be noted. A case of bilateral facial nerve dysfunction in a neonate, the offspring of a thoroughly studied kindred with known craniometaphyseal dysplasia, Is presented. The clinical and radiographic features of the disorder are discussed, including an outline of the operative management of facial paralysis.

2020 ◽  
pp. 014556132096893
Author(s):  
Dylan Jacob Cooper ◽  
Stanley Pelosi

We present a case of novel penetrating temporal bone trauma with a pintail comb causing facial paralysis. We describe a 42-year-old woman with acute facial paralysis, hearing loss, and dizziness following accidental tympanic membrane puncture. The patient underwent middle ear exploration with tympanoplasty and was found to have an intact but severely edematous facial nerve. The patient demonstrated less than 90% degeneration under electroneuronography and was treated medically without decompression, and by 6 months had exhibited complete resolution of facial nerve dysfunction with normal hearing. This case report highlights a unique cause of penetrating temporal bone trauma and supports the utility of electroneuronography in predicting the likelihood of recovery and need for decompression in patients where the facial nerve has obvious signs of trauma but remains grossly intact.


Author(s):  
Kiran Natarajan ◽  
Koka Madhav ◽  
A. V. Saraswathi ◽  
Mohan Kameswaran

<p>Bilateral temporal bone fractures are rare; accounting for 9% to 20% of cases of temporal bone fractures. Clinical manifestations include hearing loss, facial paralysis, CSF otorhinorrhea and dizziness. This is a case report of a patient who presented with bilateral temporal bone fractures. This is a report of a 23-yr-old male who sustained bilateral temporal bone fractures and presented 18 days later with complaints of watery discharge from left ear and nose, bilateral profound hearing loss and facial weakness on the right side. Pure tone audiometry revealed bilateral profound sensori-neural hearing loss. CT temporal bones &amp; MRI scans of brain were done to assess the extent of injuries. The patient underwent left CSF otorrhea repair, as the CSF leak was active and not responding to conservative management. One week later, the patient underwent right facial nerve decompression. The patient could not afford a cochlear implant (CI) in the right ear at the same sitting, however, implantation was advised as soon as possible because of the risk of cochlear ossification. The transcochlear approach was used to seal the CSF leak from the oval and round windows on the left side. The facial nerve was decompressed on the right side. The House-Brackmann grade improved from Grade V to grade III at last follow-up. Patients with bilateral temporal bone fractures require prompt assessment and management to decrease the risk of complications such as meningitis, permanent facial paralysis or hearing loss. </p>


1988 ◽  
Vol 97 (6) ◽  
pp. 675-679 ◽  
Author(s):  
Edward L. Applebaum ◽  
Arvind Kumar ◽  
Lawrence F. Berg ◽  
Mahmood F. Mafee

The recent application of arthroscopic surgical techniques to the temporomandibular joint (TMJ) has facilitated the diagnosis and treatment of TMJ disorders. However, as TMJ arthroscopy is performed more frequently, new complications are being recognized. We report three patients who developed severe otologic complications following TMJ arthroscopy. Two sustained complete or severe sensorineural hearing loss and severe vertigo from trauma to the ipsilateral ear. The third patient had complete facial paralysis from trauma to the facial nerve in the middle ear and a conductive hearing loss from incus dislocation. Complete hearing loss and facial paralysis from trauma to the main trunk of the facial nerve have not been reported previously as complications of TMJ arthroscopy.


2012 ◽  
Vol 33 (3) ◽  
pp. E14 ◽  
Author(s):  
Shaheryar F. Ansari ◽  
Colin Terry ◽  
Aaron A. Cohen-Gadol

Object Various studies report outcomes of vestibular schwannoma (VS) surgery, but few studies have compared outcomes across the various approaches. The authors conducted a systematic review of the available data on VS surgery, comparing the different approaches and their associated complications. Methods MEDLINE searches were conducted to collect studies that reported information on patients undergoing VS surgery. The authors set inclusion criteria for such studies, including the availability of follow-up data for at least 3 months, inclusion of preoperative and postoperative audiometric data, intraoperative monitoring, and reporting of results using established and standardized metrics. Data were collected on hearing loss, facial nerve dysfunction, persistent postoperative headache, CSF leak, operative mortality, residual tumor, tumor recurrence, cranial nerve (CN) dysfunction involving nerves other than CN VII or VIII, and other neurological complications. The authors reviewed data from 35 studies pertaining to 5064 patients who had undergone VS surgery. Results The analyses for hearing loss and facial nerve dysfunction were stratified into the following tumor categories: intracanalicular (IC), size (extrameatal diameter) < 1.5 cm, size 1.5–3.0 cm, and size > 3.0 cm. The middle cranial fossa approach was found to be superior to the retrosigmoid approach for hearing preservation in patients with tumors < 1.5 cm (hearing loss in 43.6% vs 64.3%, p < 0.001). All other size categories showed no significant difference between middle cranial fossa and retrosigmoid approaches with respect to hearing loss. The retrosigmoid approach was associated with significantly less facial nerve dysfunction in patients with IC tumors than the middle cranial fossa method was; however, neither differed significantly from the translabyrinthine corridor (4%, 16.7%, 0%, respectively, p < 0.001). The middle cranial fossa approach differed significantly from the translabyrinthine approach for patients with tumors < 1.5 cm, whereas neither differed from the retrosigmoid approach (3.3%, 11.5%, and 7.2%, respectively, p = 0.001). The retrosigmoid approach involved less facial nerve dysfunction than the middle cranial fossa or translabyrinthine approaches for tumors 1.5–3.0 cm (6.1%, 17.3%, and 15.8%, respectively; p < 0.001). The retrosigmoid approach was also superior to the translabyrinthine approach for tumors > 3.0 cm (30.2% vs 42.5%, respectively, p < 0.001). Postoperative headache was significantly more likely after the retrosigmoid approach than after the translabyrinthine approach, but neither differed significantly from the middle cranial fossa approach (17.3%, 0%, and 8%, respectively; p < 0.001). The incidence of CSF leak was significantly greater after the retrosigmoid approach than after either the middle cranial fossa or translabyrinthine approaches (10.3%, 5.3%, 7.1%; p = 0.001). The incidences of residual tumor, mortality, major non-CN complications, residual tumor, tumor recurrence, and dysfunction of other cranial nerves were not significantly different across the approaches. Conclusions The middle cranial fossa approach seems safest for hearing preservation in patients with smaller tumors. Based on the data, the retrosigmoid approach seems to be the most versatile corridor for facial nerve preservation for most tumor sizes, but it is associated with a higher risk of postoperative pain and CSF fistula. The translabyrinthine approach is associated with complete hearing loss but may be useful for patients with large tumors and poor preoperative hearing.


2020 ◽  
Vol 20 (1) ◽  
pp. E57-E57 ◽  
Author(s):  
Daniel M Heiferman ◽  
Esther A Cheng ◽  
Michael E Harkins ◽  
Matthew R Reynolds ◽  
Richard W Borrowdale ◽  
...  

Abstract A 37-yr-old female with prior transient left facial paralysis presented with hearing loss, headaches, and resolved transient right facial paralysis. The neurological examination demonstrated normal facial movement, left hearing loss, and left vocal cord weakness. Magnetic resonance imaging demonstrated a &gt;3 cm left paraganglioma traversing the jugular foramen. After obtaining informed consent from the patient, the tumor was embolized and then resected via a combined left postauricular infratemporal fossa and transcervical approach with cranial nerve monitoring. The ossicles were removed and the vertical segment of the facial nerve was skeletonized. The jugular bulb was identified in the hypotympanum and the petrous carotid artery was exposed. The digastric muscle was reflected inferiorly and the extratemporal facial nerve was identified. The stylomandibular ligament was transected to unlock the exposure to the infratemporal fossa. The external carotid branches were ligated. The vagus nerve and cervical sympathetic chain were infiltrated with tumor, requiring resection. The presigmoid dura and occluded jugular bulb were opened to complete the tumor resection, while preserving the medial wall. Despite anatomic preservation, the glossopharyngeal, accessory, and hypoglossal nerves were postoperatively weak and a facial paralysis recovered after 1 wk. Magnetic resonance imaging at 1 yr demonstrated a clean jugular foramen, although a thin rim of tumor remained around the petrous carotid.


2019 ◽  
Vol 5 (1) ◽  
pp. 20180029
Author(s):  
Yaotse Elikplim Nordjoe ◽  
Ouidad Azdad ◽  
Mohamed Lahkim ◽  
Laila Jroundi ◽  
Fatima Zahrae Laamrani

Facial nerve aplasia is an extremely rare condition that is usually syndromic, namely, in Moebius syndrome. The occurrence of isolated agenesis of facial nerve is even rarer, with only few cases reported in the literature. We report a case of congenital facial paralysis due to facial nerve aplasia diagnosed on MRI, while no noticeable abnormality was detected on the temporal bone CT.


2010 ◽  
Vol 37 (4) ◽  
pp. 508-510 ◽  
Author(s):  
Shigetaka Shimizu ◽  
Kumiko Yukawa ◽  
Sachie Kawaguchi ◽  
Yukari Okubo ◽  
Mamoru Suzuki

1996 ◽  
Vol 105 (1) ◽  
pp. 58-62 ◽  
Author(s):  
Clark A. Elliott ◽  
George H. Zalzal ◽  
Wendy R. Gottlieb

We reviewed 10 children who presented with facial paralysis after the onset of acute otitis media. The objective of the study was to examine the outcome of facial paralysis in children with acute otitis media treated without facial nerve decompression. Two groups were identified: 8 patients with incomplete paralysis and 2 with complete paralysis. Seven of the 8 patients with incomplete paralysis had rapid return of function after myringotomy and intravenous antibiotics. The eighth patient had delayed recovery requiring 9 months before complete return of function. The 2 patients with complete paralysis required mastoidectomy to control otorrhea and fever after initial myringotomy and antibiotics. Both patients had a prolonged recovery requiring 3 and 7 months for complete recovery. Patients with incomplete paralysis generally show rapid improvement following wide myringotomy and antibiotic treatment. A more protracted recovery may be expected in patients with complete paralysis; excellent return of function is expected when mastoidectomy without facial nerve decompression is employed.


2022 ◽  
pp. 105566562110707
Author(s):  
Elina Kapoor ◽  
Esperanza Mantilla-Rivas ◽  
Md Sohel Rana ◽  
Marudeen Aivaz ◽  
Daniela Duarte-Bateman ◽  
...  

Objective Robin Sequence (RS), characterized by micrognathia, glossoptosis, and upper airway obstruction, is an increasingly recognized diagnosis. An effective surgical intervention is mandibular distraction osteogenesis (MDO). This study analyzes published evidence regarding facial nerve dysfunction (FND) associated with MDO. Design and Setting According to PRISMA guidelines, a systematic review was carried out with databases queried in June 2019 using MESH terms, or equivalent terms, as follows: “distraction osteogenesis” and “Robin Sequence”. A review of original Spanish and English articles, were included. Outcome measures included the prevalence of FND; the affected branches; the rate of permanent vs. transient FND; the use of an internal vs. external device; the daily distraction rate; and finally, the overall distraction length. Subsequently, a meta-analysis was conducted to collate results regarding the prevalence of FND and the factors associated with it. Results Of 239 unique studies identified, 19 studies with 729 patients met inclusion criteria; 52 patients developed FND after MDO. A random-effects meta-analysis yielded a pooled prevalence of FND of 6.40%, with moderately heterogeneous studies (I2 = 41%, τ2 = 0.006). Marginal mandibular nerve involvement was most commonly noted. Nine studies reported transient FND, six permanent, one both, and two unspecified. Internal distractors were used in 8 studies and external in 3 and both in 2. Distraction rate was 1.00 to 2.00 mm/day and total distraction length ranged from 13.00 to 22.3 mm. Sample size was the only parameter inversely associated with rate of FND (p = 0.04). Conclusion This analysis of FND associated with MDO for patients with RS demonstrates a lack of consistent documentation. MDO-associated FND does not appear to be uncommon, and permanent dysfunction can occur. This review underscores the importance of thorough documentation to elucidate the mechanism of FND.


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