a6 6342508 213805000, THE SOURNAL OF ‘RaMowenowuLen PRACTICE Copyrant@ 2007 by CHROMA, Io, aout ecdved Januay 3, 2006 eased ‘ramuseritecaved Seplmber 252006 aczaptes Seplrbr 27,2006 ‘rss or coraspendose ‘t Cosme Gay-Esota Cerotéaco Tekren cana 2, Despach 170 (8022 Barcelona, Span Ermat coapaud edu CASE REPORT Nervus Intermedius Neuralgia: A Case Report Rui Figueiredo, D. Jeffrey P. Okeson, D.M.D. ; Eduardo Vazquez-Delgado, D.D.S., M.S.; ‘osme Gay-Escoda, D.D.S., M.D., Ph.D. ABSTRACT: Nervus intermedius neuralgia (NIN) is an uncommon disorder that affects a sensory branch of the facial nerve. This condition usually provokes a very intense and stabbing pain localized in the depth of the ear canal. Due to the close anatomical proximity, temporomandibular joint (TM) pathologies should be included in the differential diagnosis. The treatment of NIN has not been estab- lished, although it seems reasonable that the therapeutic approaches used in other more common cran- iofacial neuralgias, such as trigeminal neuralgia, should be effective. In this paper, the authors present a case report of a female patient diagnosed with NIN who was successfully managed with pharmacologi- cal treatment, De. Rui Figueiredo received his DDS degre in 2002 from the Instituto Superior ddeCiéncias da Sade, Oporte, Portugal. 12008, he finished the Master's degree rogram ioral surgery and implantology lathe School of Dentists ofthe University of Barcelona, Hes curventy an associate professor of oral surgery and «professor ofthe oral surgery and inplanttogy Masters degree program athe same Dr. Eduardo Vazquer-Delgado received his DDS. degree in 1999 from the Coege of Dentistry, University of Barcelona Spain. In 2003. he obtained his MS. degree in orofacial pain from the Collegeof Denistry, University of Kentucky. He iseurrently a professor of the TMD and Orofacal Pain Unit ofthe ‘oral surgery ad inplantology Master's degre program, University of Barcelona, ‘and member ofthe TMD and Orefacial Pain Unt, Teknon Medical Center Barcelona 213 he assessment of orofacial pain is generally a chal- I lenge even for the most experienced clinicians, since multiple clinical entities usually need to be ruled out in order to establish a final diagnosis. When one considers the amount of anatomical structures included in the cranio-maxillofacial area and the complexity of the pathophysiological mechanisms of pain referral, it can be concluded that only a multidisciplinary approach to oro- facial pain syndromes is effective.! The “International Classification of Headache Disorders of the International Headache Society (IHS), which is regarded as the one of the most reliable and widely used classifications for diag nosing orofacial pain disorders, embraces an extensive amount of clinical entities that manifest symptoms in the craniofacial structures. Among these, nervus intermedius neuralgia (NIN) (subgroup 13.3 of the IHS classification) is an extremely rare condition thought to be similar to trigeminal neuralgia but involving another cranial nerve (a sensory branch of the facial nerve that innervates the external ear canal) (Table 1), To date, there are very few reports published in the scientific literature, which makes the incidence of NIN in the general population unknown. Patients usually report a stabbing pain deep in the auricu- lar canal that can last seconds or minutes and that remits spontancously.* These episodes occur for no apparent reason, since the patient is unable to relate the pain withNERVUS INTERMEDIUS NEURALGIA FIGUEIREDO ET AL. Table 1 Diagnostic Criteria for NIN Accor Classification of Headache Disorders of the IHS? "Pain paroxyems of intermitient occurrence, lasting for seconds or minutes, in the dapih ofthe ea B, Presence of a tigger area inthe posterior wall of the auditory canal C. Not attributed to another disorder. any physical activity. Initially, a thorough clinical exam- ination of the middle ear should be performed in order to exclude any lesion or pathological condition of the oto- logical structures.*7 Subsequently, a complete neurologi- cal and dental examination is paramount as several other conditions need to be considered in the differential diag nosis, including carcinoma of the nasopharynx, temporal bone, or external auditory canal, Eagle’s syndrome and, TMJ disorders? (Table 2). Dentists should be aware of neura that present with symptoms that are in close anatomical proximity to the temporomandibular joints (TMI), so that the proper diagnosis can be established leading to proper treatment selection, In fact, the patient presented in this case was referred to us by her dentist to assess a pain in the TMJ The aim of this paper is to present a case report of NIN and to review the literature on the subject Case Report A 68-year-old female caucasian patient was seen in the ‘TMJ and Orofacial Pain Unit of the Oral Surgery and Implantology Department of the School of Dentisiry of the University of Barcelona, She was referred by her den- tist to evaluate a high intensity pain in her right TMJ that had been present for more than 15 years. The patient reported an electrical, stabbing pain, localized in the depth of her right ear, with an intensity of 10/10 on a ‘Visual Analog Scale (VAS) and lasting two to three sec~ onds. She reported one to two episodes of this pain per week. Shee had been seen by her general practitioner and by several different specialists for this pain, including an otolaryngologist, traumatologist, and an oral and max- illofacial surgeon. She reported that their clinical and radiographic examinations found no relevant abnormali- ties that could explain the pain, ‘The patient's past medical history revealed high blood pressure, dyslipemia, arthritis, and lumbar hernia t 214 THE JOURNAL OF CRANIOMANDIBULAR PRACTICE, present med (Cozaar Plus 50 mg+12.5 mg, Esteve, Barcelona, Spain), and acetylsalicylic acid (Adito 100 mg, Bayer, Barcelona, Spain). Medication trials with ibuprofen (Algiasdin 600 mg, Esteve, Barcelona, Spain) or acetylsalicylic acid (Aspirina 500 mg, Bayer, Barcelona, Spain) failed to con- trol her pain symptoms. Likewise, psychological therapy was ineffective The cranial nerve examination was within normal limits, as well as mandibular and cervical ranges of motion, Palpation of the cervieal and masticatory mus- cles did not originate any pain or discomfort. There was, also no pain when palpating the TMJ. An asymptomatic reciprocal click was present in the right TMJ. The intrao- ral tissues showed no signs of pathology. However, upon examination of the posterior wall of the right auditory ‘canal, a trigger zone was found (Figure 1), Pressing on the area with a cotton tip reproduced the patient's pain complaints. A panoramic radiograph (Figure 2) and a cranial computer tomography (CT) were done to exclude the presence of any intracranial pathology (Figure 3). These imaging exams were within normal limits, Based on the patient's history and clinical examina- tion, a diagnosis of NIN was established. Initial treatment began with educating the patient about the condition. A series of blood tests were requested in order to obtain baseline parameters before the administration of any medication, Medical management began with 300 mg of, oxcarbazepine (Trileptal, Novartis Pharmaceutical, Barcelona, Spain) before bedtime. The patient was instructed to increase by one tablet of oxcarbazepine every four days until a 900 mg daily dose was reached. She was also advised to contact the clinic if she experi- enced any major side effects such as tremors, diarrhea, ion, drowsiness, rash, and fatigue, among others. After six weeks of treatment, the patient reported a substantial reduction in pain intensity (2/10 VAS) and frequency (one episode a week). She was taking 300 mg JULY 2007, VOL. 25, NO. 3FIGUEIREDO ET AL. NERVUS INTERMEDIUS NEURALGIA Table 2 Clinical Features of Several Pathologies Included in the Differential Diagnosis of Nervus Intermedius Neuralgia CLINICAL FAN IMAGING | SPECIAL ENTITIES | iwrensrry | puratioy | Location | quatiry | TRIGGER | EXAMS | FEATURES External ctiis | Variable | Constant | Ea iroat | Dall achy | Movement of | Normal Tid fever ear as the pina or drainage tragus ofthe idle tts (| Vaisble | Constat Ea, throat | Dull acy Absent Normal Fever, earings ar drainage Tniracerebral | Nildto ‘Variable | Fosal or | Throbbing [Absent Presence of | Tneteasing pin wit ‘tumour ©) moderate generalized | orsteady tumour head movements or valalva manoeaver. Sudden nse. Rapid ‘worsening Short periods of severe headache, ltharey and Wansicnt cal defi TM dhorders | Mildto | Constant] TM, car Sfp, acy | Tendemessin | Ta some cases | Associated with 210,15 moderate the TMI area | presence of | mandibular TM disk | movement splacement sherations Myofascial pain | Nt Constant | Ear affeiod [Dall acty | “Trigger Normal Tees wih disorders ‘moderate muscle point” nodule muscle movement or 9,11) palpation Eagle's syndrome | Mildto Constant | Thro ew, | Stabbing, [Tonsil | Falarged | Palyation of tie (213) moderate mastoid | achy fossa stylid stylid process in the region process | tonsllar fossa Bamsay Hunt [Severe Constant Avcitory | Sharp, [Absent ‘Nonna Herpetis eruptions syndrome canalcar | subbing facil palsy ay) lobe, soft pala and fongue Chasieal Seve Seconds to | Postenor | Elecical, | Pain tagered | Normal | Swallowing ‘lossopharyngeal ‘minutes | tongue, sharp, | byeating and chewing, taking, neuralgia pharm, | stabbing | swallowing ‘coughing and/or ai tonsa fossa ‘yawning produce orca pain NIN Severe Sexonds or | Ear ‘Hectrical, | Posterior wall | Normal | Presence of tiezer es) ‘minutes sap, | of auditory zea in ary canal stabbing | canal of oxcarbazepine in the morning and 600 mg in the 12. The patient reported no side effects from the ‘medication, Given the partial but favorable response and lack of adverse side effects, the decision was made to increase the daily dose of oxcarbazepine to 600 mg BID. ‘The patient was re-evaluated four weeks later. She reported that her current pain level was 1/10 (VAS). She had experienced only one pain episode since the last follow-up visit. There were no adverse side effects from the medication, The patient was extremely satisfied with the treatment outcome. She was then told to gradually JULY 2007, VOL. 25, NO. 3 decrease her daily dose of oxcarbazepine to 300 mg BID. She was also advised to contact the clinic if she experi- enced a worsening of her pain symptoms and was sched- uled for a follow-up visit in six weeks. The patient was re-evaluated four months after treatment onset. She reported that her current pain level was 1/10 (VAS) and that her pain frequency was one episode per month. There were no side effects from the medication, The patient was instructed to continue with her present medical treatment (oxcarbazepine 300 mg BID), and she was scheduled for a follow-up visit in four months. THE JOURNAL OF CRANIOMANDIBULAR PRACTICE 215NERVUS INTERMEDIUS NEURALGIA FIGUEIREDO ET AL, Figure 1 Detection ofthe trigger are using a cotton tip in the posterior wall of the auditory canal Discussion Nervus intermedius neuralgia (NIN) has a very low incidence in the neral population and therefore, ean be easily misdiagnosed even by the most skilled clinicians. For that reason, clinical reports on this subject have con- siderable clinical relevance. The current report describes, a female patient with a 15 year history of undia pain, despite heing examined by a n fessionals. The pathophysiological mechanisms of this neuralgia are still unclear.* Some authors state that this pathology ted by the cross compression of the nerve at its, Imber of health pro- central-peripheral myelin junction, Based on the surgical findings, it seems that NIN may be originated by vascular compression of several anatomical structures. In the cur 216 THE JOURNAL OF CRANIOMANDIBULAR PRACTICE rent case report, this was not assessed, since no surgical procedures or imaging exams to confirm such etiology were performed, The diagnosis is based on several clinical features. The pain paroxysms are always unilateral, intermittent, and last from seconds to minutes. A trigger point located on the posterior wall of the auditory canal seems to be criti- cal in its diagnosis, since it allows the clinician to exclude other pathologies. The radiographic exams usu- ally do not provide relevant information. A number of case reports, similar to this one, show spotless comple: ‘mentary exams.™!® An anesthetic block of the area is also advocated by some authors as a diagnostic tool." However, in this patient, this procedure was not helpful due to the long pain free periods between the attacks. A pharmacological approach should always be attempt- cd before any surgical procedures are considered for the treatment of NIN. Thus far, there is a lack of high quality clinical trials (randomized clinical trials) that would sup- port the use of any specifie form of pharmacological ther- apy for the treatment of NIN, Nonetheless, clinical experience indicates that those medications used for the treatment of neuropathic pain seem to be at least mod- estly effective in the treatment of NIN. We were unable to arding the specific conventional phar macological treatment of NIN, Accordingly, we applied a iment protocol as is used for other find any study rey neuralgias such as trigeminal neuralgia, Carbamazepine is considered to be the treatment of choice in such disor- ders.4i01S17 Its efficacy has been proven in many clinical trials, although several side effects have been reported, including blood dyscrasias, allergic skin reactions, liver and renal function alterations, and hyponatremia, which may have serious clinical consequences.'*” For this reason, in recent years, treatment with oxcarbazepine has Figure 2 Panoramic radiograph, JULY 2007, VOL. 25, NO. 3FIGUEIREDO ET AL. NERVUS INTERMEDIUS NEURALGIA Figure 3 Axial view ofthe eranial CT sean, been advocated, since this medication provides a similar and a better profile regarding safety and tolerability." The patient in this report responded in a very favorable manner to this medication. In some instances, it is possible to discontinue the medication without recurrence of the symptoms, although, this is quite unusual. Most patients are forced to continue with a long lasting pharmacological treatment." In order to avoid side effects, the dose should be diminished in a clinical effica progressive way until a minimum effective dosage is obtained, Nonetheless, the patient must be under close surveillance, with routine blood samples performed every three to six months. depending on the patient’s age and medical history Several surgical approaches have also been described to manage this neuralgia with satisfactory results.*"** Even so, microvascular decompression and thizotomies are options that should only be considered when pharma- cological treatments provide inadequate pain control or intolerable side effects.” A report by Rupa, et al.! showed that these surgical procedures have an overall success rate of 72% in providing pain relief. Nevertheless, they also report that several side effects should be expected, such as decreased salivation, lacrimation, and taste."* However, the results described by Rupa, et al.!® should be taken with caution since the study sample was relatively heterogeneous and included several distinct and unclear clinical entities. This case report suggests that NIN, although rare, should be considered in the differential diagnosis of oro facial pain disorders. It also indicates that oxcarbazepine JULY 2007, VOL. 25, NO. 3 may be helpful in the management of NIN. Future studies are needed to provide more information regarding the prevalence, etiology, and treatment of this disorder. References 1. Shara ¥: Oral pain: Wall P, Melack es, Testo of pai. ed London: Chachi Livingstone, 1994; 563-582 2. The iemational lasifcton for eadacke disorders, Cephal 2008; 24 Suppl I-16, 4. Potarochs Digo M. Ragin Sebati J. Mil Massact M. Burguer ‘ere J: Neuralgia pati de item, Av Odontol 4. Year EA Yop: Nersitrmes neural an uncommon pin sy tome with an uncommon etilgy-J Pan pion Mgnt 2001907. Ps 5. Baym GW: News intermedia neuralgia (Hunt). Cephal 194; 4:71-78 6. AlerazN. King WA Wackym PA: Endoscopy diag neurtomy ofthe ers ncrmediaefor penile neralga, targa! Head Nek Surg ooo 131s 8 Lovely Tn Pl Serial management of genic 1. Bare RG: Pin experience in chien: development and clinical charters tis In: Wall, Melzack R, els, Textbook of pai. rd ed. Londons {Corel Livnpaene, 1983.79.75, 8, Chery NI, Portnoy RK: Cancer pain: principles of assessment and sy tomes in; Wall P. Metzach Reeds Festool of pn ded London ‘Churchil Livingstone. 1984.78 10, Okemo pas, the, Chisago: Quintessence Pubshing 1, Petrrocha-Digo M: Door oof. xan dlgndnicoytratamiem, Tacks Masson, 19 12, Rechtwog JS, Was MK: Eagle’ syn Too bate Ste T185-54, 18. Carina EM erg. Am} me: a review. Am J Otlargl Dr. Jeffrey P. Okeson received his D.M.D. degree in 1972 from the College of Denustry, University of Renuck. He Jotned the faculy ofthe University of Rentcky in 1974. Presently, he is professor and director af ‘the Orofacial Pain Cente atthe same faculty, which he established in 1977. Dr. Okeson has written manyclinical and research articles ad is ‘hor of several textbooks tha are widely used in dental schools and ‘orofacial pain programs throughout the world, Dr. Cosme Gay-Escoda received hie MD. degree in 1974 and his DDS. degree in 1976. In 1986, he fished his PhD. degree Later, he joined the School of Demy ofthe Universiy of Barceloma where he ‘as named Dean fra iyo peri (198911998), Presently, hei professor and chairman ofthe Oral Su gery and Implantooey Deparment at the same faculty, and he develops his clinical activities as ‘an oral an manilofcial surgeon inthe Teknon Medical Centr of | Barcelona. Prof Gay-Escoda has writen several books and more than 200 clinical and research apes, mainly inthe felds of oral and mac iofacialsurgers and implanvlogy. THE JOURNAL OF CRANIOMANDIBULAR PRACTICE 217