Sensorineural hearing loss and vertigo as first symptoms of Arnold-Chiari malformation

Introduction: Arnold-Chiari malformation is a congenital malformation of the skull in which the cerebellum and the medulla are pushed into the upper cervical canal outside the foramen magnum. Chiari malformation can manifest with headache, ataxia, tinnitus and audio-vestibular symptoms that generally worsen with time. A correct diagnosis leads to timely surgical intervention. Diagnosis of the Arnold-Chiari malformation is confirmed with MRI. Treatment generally involves surgical decompression of the surrounding spinal tissue. Case presentation: We present a 15-year-old boy, with sudden symptoms of dizziness, vertigo, tinnitus and hearing loss in the right ear, brought on during exercise. Dizziness, tinnitus and vertigo disappeared after few days but a hearing loss remained. He denied having headaches. He has hypertension and that was considered as the cause of the new symptoms. Complete audio-vestibular examination was performed and sensorineural hearing loss on the right ear was revealed. MRI brain scan was made and caudal herniation of the medulla oblongata and cerebellar tonsils through foramen magnum (by 16 mm) was discovered. Herniation was more pronounced on the right side, medulla oblongata was pushed forward, and the cerebrospinal fluid space in that level was reduced. Conclusion: Arnold-Chiari malformation can present itself with numerous symptoms. Hearing loss, tinnitus and vertigo need to be considered as a possible sign of this disorder

Sensorineural hearing loss and vertigo as first symptoms of Arnold-Chiari malformation

Introduction: Arnold-Chiari malformation is a congenital malformation of the skull in which the cerebellum and the medulla are pushed into the upper cervical canal outside the foramen magnum. Chiari malformation can manifest with headache, ataxia, tinnitus and audio-vestibular symptoms that generally worsen with time. A correct diagnosis leads to timely surgical intervention. Diagnosis of the Arnold-Chiari malformation is confirmed with MRI. Treatment generally involves surgical decompression of the surrounding spinal tissue. Case presentation: We present a 15-year-old boy, with sudden symptoms of dizziness, vertigo, tinnitus and hearing loss in the right ear, brought on during exercise. Dizziness, tinnitus and vertigo disappeared after few days but a hearing loss remained. He denied having headaches. He has hypertension and that was considered as the cause of the new symptoms. Complete audio-vestibular examination was performed and sensorineural hearing loss on the right ear was revealed. MRI brain scan was made and caudal herniation of the medulla oblongata and cerebellar tonsils through foramen magnum (by 16 mm) was discovered. Herniation was more pronounced on the right side, medulla oblongata was pushed forward, and the cerebrospinal fluid space in that level was reduced. Conclusion: Arnold-Chiari malformation can present itself with numerous symptoms. Hearing loss, tinnitus and vertigo need to be considered as a possible sign of this disorder

Rinogeni meningitis uzrokovan kongenitalnim kolesteatomom apeksa piramide: simultano kirurško liječenje transotičkim i transsfenoidnim pristupom

A 66-year-old male patient was admitted due to high fever, severe headaches and disturbance of consciousness. Meningitis was confirmed by lumbar puncture and intravenous antimicrobial therapy was started. Since he had undergone radical tympanomastoidectomy 15 years before, otogenic meningitis was suspected, so the patient was referred to our department. Clinically, the patient manifested watery discharge from the right nostril. Microbiological analysis verified Staphylococcus aureus in a cerebrospinal fluid (CSF) sample acquired by lumbar puncture. Radiological work-up, including computed tomography and magnetic resonance imaging scans, showed an expanding lesion of the petrous apex of the right temporal bone disrupting the posterior bony wall of the right sphenoid sinus with radiological characteristics indicating cholesteatoma. Those findings confirmed rhinogenic meningitis caused by expansion of the petrous apex congenital cholesteatoma into the sphenoid sinus, allowing nasal bacteria to enter the cranial cavity. The cholesteatoma was removed completely by the simultaneous transotic and transsphenoidal approach. Since the right labyrinth was already non-functional, there was no surgical morbidity after labyrinthectomy. The facial nerve remained preserved and intact. The transsphenoidal approach enabled removal of the sphenoid portion of the cholesteatoma and two surgeons met together at the level of the retrocarotid segment of the cholesteatoma, completely removing the lesion. This case represents an extremely rare condition in which a petrous apex congenital cholesteatoma expanded through the petrous apex to the sphenoid sinus, causing CSF rhinorrhea and rhinogenic meningitis. According to available literature, this is the first case of petrous apex congenital cholesteatoma causing rhinogenic meningitis successfully treated with the simultaneous transotic and transsphenoidal approach.Bolesnik u dobi od 66 godina primljen je zbog visoke temperature, jake glavobolje i poremećaja svijesti. Lumbalna punkcija potvrdila je meningitis i započeta je intravenska antibiotska terapija. Budući da je 15 godina ranije kod bolesnika rađena radikalna timpanomastoidektomija, postavljena je sumnja na otogeni meningitis te je upućen na našu Kliniku. Nakon primitka je uočena desnostrana rinolikvoreja, a ponovljenom lumbalnom punkcijom u likvoru je izoliran Staphylococcus aureus. Radiološka obrada uključujući kompjutoriziranu tomografiju i magnetsku rezonancu pokazala je ekspanzivnu leziju vrha piramide desne temporalne kosti s destrukcijom stražnje stijenke sfenoidnog sinusa i radiomorfološkim osobinama kolesteatoma. Ovi nalazi potvrdili su da se radi o rinogenom meningitisu koji je uzrokovan širenjem kongenitalnog kolesteatoma vrha piramide u sfenoidni sinus, što je omogućilo prodor bakterija iz nosa u endokranij. Kolesteatom je u cijelosti odstranjen simultanim kombiniranim transotičkim i transsfenoidnim pristupom. Budući da je desni labirint od ranije bio nefunkcionalan, nije bilo kirurškog morbiditeta nakon labirintektomije. Očuvani su integritet i funkcija ličnog živca. Transsfenoidni pristup omogućio je odstranjenje sfenoidnog dijela kolesteatoma, a oba operatera susrela su se na razini retrokarotidnog segmenta kolesteatoma koji je tako odstranjen u cijelosti. Ovaj bolesnik predstavlja iznimno rijedak slučaj kod kojega se kongenitalni kolesteatom vrha piramide proširio u sfenoidni sinus uzrokujući rinolikvoreju i rinogeni meningitis. Prema dostupnoj literaturi, ovo je prvi takav slučaj koji je ujedno uspješno liječen simultanim transotičkim i transsfenoidnim pristupom

CLINICAL AND PATHOPHYSIOLOGICAL PATTERNS OF OTITIS EXTERNA AND OVERVIEW OF PROBLEMATIC CASES

Upala vanjskog uha katkad je važan klinički problem, ponajprije u slučajevima neuspješnog odgovora na uobičajene terapijske postupke, dugotrajnijeg tijeka bolesti ili u slučaju učestalih recidiva s pojavom komplikacija. U tim slučajevima potrebno je obratiti posebnu pozornost na mogućnost pogreške u dijagnostici, nepravilan slijed terapijskih postupaka ili na postojanje neprepoznatih čimbenika, bilo predispozicije pacijenta, patogenosti uzročnika ili drugih razloga. U radu donosimo prikaz slučaja 84-godišnjeg pacijenta s kompliciranom upalom vanjskog zvukovoda, kratak pregled fiziologije zvukovoda i patofiziologije upale, etioloških čimbenika, dijagnostike i terapijskih postupaka te pregled mogućih postupaka u težim, tvrdokornijim slučajevima upale.Otitis externa can sometimes present itself as a difficult clinical problem, in cases such as unsuccessful outcome of usual therapy, prolonged duration of symptoms or frequent recurrencies with local or systemic complications. In these cases more attention should be focused on possible errors made in establishing the right diagnosis, incorrect sequence of applying therapeutical measures, or presence of unrecognized problems, including individual patient factors, characteristics of the pathogen, or other. In this article we present a case report of a 84-year-old patient with complicated otitis externa, together with a short review of physiology, patophysiology, diagnostic and therapeutical measures in external otitis, and suggest a possible clinical approach in the management of problematic cases

Temporal bone meningocele presenting as a secretory otitis media

Temporal bone meningocele is an uncommon condition in which middle fossa meninges prolapse into the middle ear through a tegmen defect. These bone defects may be spontaneous or acquired, resulting from head trauma or ear surgery. Some of the most common symptoms include conductive hearing loss, headache, otorrhea and recurrent acute otitis media. Rare but more severe complications may be otogenic meningitis or cerebral abscess

CLINICAL AND PATHOPHYSIOLOGICAL PATTERNS OF OTITIS EXTERNA AND OVERVIEW OF PROBLEMATIC CASES

Upala vanjskog uha katkad je važan klinički problem, ponajprije u slučajevima neuspješnog odgovora na uobičajene terapijske postupke, dugotrajnijeg tijeka bolesti ili u slučaju učestalih recidiva s pojavom komplikacija. U tim slučajevima potrebno je obratiti posebnu pozornost na mogućnost pogreške u dijagnostici, nepravilan slijed terapijskih postupaka ili na postojanje neprepoznatih čimbenika, bilo predispozicije pacijenta, patogenosti uzročnika ili drugih razloga. U radu donosimo prikaz slučaja 84-godišnjeg pacijenta s kompliciranom upalom vanjskog zvukovoda, kratak pregled fiziologije zvukovoda i patofiziologije upale, etioloških čimbenika, dijagnostike i terapijskih postupaka te pregled mogućih postupaka u težim, tvrdokornijim slučajevima upale.Otitis externa can sometimes present itself as a difficult clinical problem, in cases such as unsuccessful outcome of usual therapy, prolonged duration of symptoms or frequent recurrencies with local or systemic complications. In these cases more attention should be focused on possible errors made in establishing the right diagnosis, incorrect sequence of applying therapeutical measures, or presence of unrecognized problems, including individual patient factors, characteristics of the pathogen, or other. In this article we present a case report of a 84-year-old patient with complicated otitis externa, together with a short review of physiology, patophysiology, diagnostic and therapeutical measures in external otitis, and suggest a possible clinical approach in the management of problematic cases

Multifocal metachronous occurence of different hystologic sinonasal-type papilloma: a case report

Introduction: Sinonasal-type papilloma is a very rare tumor, most commonly connected with the sinonasal space, and very rarely with the middle ear. Primary tumors of the temporal bone are extremely rare and only 28 cases have been described in literature, with additional 29 cases of tumor spreading from the sinonasal tract to the temporal bone. Case presentation: We discuss the case of a 49-year-old woman who had a primary right-sided exophytic form of the sinonasal papilloma of the middle ear, which led to right-sided hearing loss, aural fullness, and otorrhea. During postoperative CT and MRI follow-up one year after surgery, a sinonasal oncocytic-type papilloma was discovered in the sphenoid sinus. To our knowledge, this is the first described case of histologically two different primary sinonasal-types of papilloma in a patient. Common presenting symptoms associated with sinonasal papilloma of the middle ear can be easily misdiagnosed with chronic otitis media or Eustachian tube dysfunction. Although primarily benign, sinonasal papillomas are locally aggressive and pose a risk of recurrence and malignant transformation. Therefore, surgery remains the treatment of choice with necessary long-term follow-up, to detect relapse or even a completely new tumor in that area

Usporedba postoperativnog praga sluha nakon stapedotomije ovisno o tipu proteze: retrospektivna analiza

Stapes surgery is generally performed to treat otosclerosis, and there are numerous surgical techniques and prosthesis materials available. Critical evaluation of postoperative hearing outcomes is crucial for identification and further improvement of treatment options. This study is a non-randomized retrospective analysis of hearing threshold levels before and after stapedectomy or stapedotomy in 365 patients during a twenty-year period. The patients were classified into three groups depending on the prosthesis and surgery type: stapedectomy with Schuknecht prosthesis placement and stapedotomy with either Causse or Richard prosthesis. The postoperative air-bone gap (ABG) was calculated by subtracting the bone conduction pure tone-audiogram (PTA) from the air conduction PTA. Hearing threshold levels were evaluated preoperatively and postoperatively from 250 Hz to 12 kHz. The results showed air-bone gap reduction <10 dB in 72% patients, 70% of patients, and 76% of patients using Schucknecht’s prosthesis, Richard prosthesis, and Causse prosthesis, respectively. The results did not differ significantly between three prothesis types. Choice of prosthesis should be made individually for each patient, but surgeon competency is still the most important outcome variable, regardless of prosthesis type.Stapedotomija ili stapedektomija su metode izbora za liječenje otoskleroze, a postoji velik broj proteza koje se koriste u obje kirurške tehnike. Konstantna evaluacija postoperativnih ishoda sluha ključna je za vrednovanje najboljeg tipa i materijala za korištenih u kirurškom liječenju otoskleroze. Ovo je istraživanje nerandomizirana retrospektivna analiza pragova sluha prije i nakon stapedektomije ili stapedotomije u 365 pacijenata tijekom desetogodišnjeg razdoblja. Pacijenti su na temelju tipa operacije i proteze svrstani u tri grupe; stapedektomija s postavljanjem Schuknechtove proteze, stapedetomija s Causse ili Richardovom protezom. Postoperativni ABG izračunat je oduzimanjem BC PTA od AC PTA. Tonskim audiogramom su evaluirani pragovi sluha prijeoperativno i postoperativno u rasponu od 250 Hz do 12 kHz. Rezultati pokazuju smanjenje zračno-koštanog prozora na <10 dB u 72% pacijenata s Schucknecht protezama, dok je u grupi pacijenata s Richardovim protezama takvih pacijenata bilo 70%, a u grupi pacijenata s Causse protezama 76%. Rezultati su bili slični za sve tri promatrane opcije. Odluka o tipu operacije i proteze trebala bi biti individualna za svakog pacijenta, a ishod operacije ne ovisi o tipu proteze

Long-Term Functional Outcomes after 10 Years of Bilateral Cochlear Implantat Use

The aims were to determine the benefit of bilateral cochlear implantation in a 20 years old patient implanted in Croatia on hearing and speech development. The male patient, after 10 years of deafness, got cochlear implants Med-EL Combi 40+ on both sides in one-stage surgery. The etiology of his deafness was posttraumatic meningitis. Auditory capacity and speech recognition tests were performed for both ears separately and together. Average hearing level on the right ear with right cochlear implant switched on started at 62 dB 1 month after the cochlear implantation and was on 55 dB after 10 years. Average hearing level on the left ear with left cochlear implant switched on started at 55 dB 1 month after the cochlear implantation and was on 32 dB after 10 years. Average hearing level on the both ears with 2 cochlear implants switched on started at 35 dB 1 month after the cochlear implantation and was on 27 dB after 10 years. Long- -term functional outcomes with bilateral cochlear implantation provides advantages over unilateral implantation including improved hearing level, speech perception in noise and improved sound localization